618 results on '"Dermatitis, Exfoliative pathology"'
Search Results
2. Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients.
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Montero-Menárguez J, Amat Samaranch V, Puig Sanz L, Ruiz-Villaverde R, Arias-Santiago S, Larrea García M, Ruiz Genao D, Ferrán M, Schneller-Pavelescu L, Romero Ferreiro C, and Rivera Díaz R
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- Humans, Male, Retrospective Studies, Female, Middle Aged, Spain epidemiology, Aged, Adult, Child, Adolescent, Child, Preschool, Young Adult, Aged, 80 and over, Dermatitis, Exfoliative etiology, Dermatitis, Exfoliative pathology, Treatment Outcome, Adrenal Cortex Hormones therapeutic use, Infant, Pityriasis Rubra Pilaris pathology, Pityriasis Rubra Pilaris drug therapy
- Abstract
Introduction: PRP is a rare entity of unknown etiopathogenesis. Lack of management guidelines makes it a challenge for clinicians., Objective: To add our experience to increase evidence about PRP., Methods: We performed a retrospective, descriptive and multicentric study of 65 patients with PRP, being the largest European case series of patients with PRP., Results: PRP was more frequent in male patients with an average age of 51 years, but erythrodermic forms presented in older patients (average age 61 years). Six (75%) paediatric patients and ten (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. On the contrary, 26 (68%) erythrodermic patients required biologic therapy as last and effective therapy line requiring an average of 6.5 months to achieve complete response., Conclusion: Our study showed a statistical difference in terms of outcome and response to treatment between children or patients with limited disease and patients who develop erythroderma., (Copyright © 2024 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2024
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3. Erythroderma-like crusted scabies in an immunocompetent patient after systemical corticosteroids.
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Ren YK, Yuan HJ, Sun W, Lang XQ, Liu HY, and Guo SP
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- Humans, Adrenal Cortex Hormones therapeutic use, Male, Female, Immunocompetence, Scabies drug therapy, Scabies pathology, Dermatitis, Exfoliative pathology, Dermatitis, Exfoliative drug therapy
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- 2024
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4. Erythroderma: clinical and etiological study of 88 cases seen in a tertiary hospital over 25 years.
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Kondo RN, Samesima E Singh B, Araújo MCP, Ferreira VP, Marani JA, and Gon ADS
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- Humans, Female, Male, Middle Aged, Adult, Aged, Young Adult, Adolescent, Aged, 80 and over, Retrospective Studies, Brazil epidemiology, Child, Child, Preschool, Dermatitis, Exfoliative pathology, Dermatitis, Exfoliative etiology, Dermatitis, Exfoliative epidemiology, Tertiary Care Centers
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- 2024
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5. Rapid and successful effects of combining dupilumab with ultraviolet B radiation therapy in the treatment of Papuloerythroderma of Ofuji.
- Author
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Chen F, Wen H, and Sun Q
- Subjects
- Humans, Skin Diseases, Papulosquamous pathology, Dermatitis, Exfoliative pathology, Ultraviolet Therapy
- Abstract
Papuloerythroderma of Ofuji (PEO) is an uncommon disease characterised by widespread erythroderma composed of intensely pruritic solid papules coalescing into plaques sparing the skin folds (deck-chair sign). The pathogenesis of PEO remains unclear, although T helper (Th) 2 and Th22 cells may play an important role. Dupilumab is an interleukin (IL)-4 receptor α-antagonist that effectively reduces Th2 responses, which has drawn increasing attention in the treatment of PEO patients. Here, we reported a successful case of dupilumab treatment in combination with ultraviolet B (UVB) radiation therapy, which is well known and effective for chronic itch. The patient had a significant decrease in visual analogue scale (VAS) score and eosinophil after only 1 week of treatment, which may be due to the combination effect., (© 2023 Australasian College of Dermatologists.)
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- 2023
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6. HLA-DR Helps to Differentiate Erythrodermic Cutaneous T-cell Lymphoma from Erythrodermic Inflammatory Dermatoses in Flow Cytometry.
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Sun J, You R, Lyu B, Li X, Gao Y, Wen Y, Qu C, and Wang Y
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- Humans, Retrospective Studies, Flow Cytometry, CD4 Antigens, HLA-DR Antigens, Dermatitis, Exfoliative pathology, Mycosis Fungoides, Skin Neoplasms pathology, Lymphoma, T-Cell, Cutaneous diagnosis, Lymphoma, T-Cell, Cutaneous pathology
- Abstract
Differential diagnosis of erythroderma is challenging in dermatology, especially in differentiating erythrodermic cutaneous T-cell lymphoma from erythrodermic inflammatory dermatoses. This study retrospectively reviewed the peripheral blood flow cytometric results of 73 patients diagnosed with erythroderma at Peking University First Hospital from 2014 to 2019. The flow cytometry antibody panel included white blood cell markers, T-cell markers, B-cell markers, T-cell activation markers, and T helper cell differentiation markers. Features of the cell surface antigens were compared between 34 patients with erythrodermic cutaneous T-cell lymphoma and 39 patients with erythrodermic inflammatory dermatoses. The percentage of HLA-DR+/CD4+T cells was the most pronounced marker to distinguish erythrodermic cutaneous T-cell lymphoma from erythrodermic inflammatory dermatoses, with a threshold of 20.85% (sensitivity 96.77%, specificity 70.37%, p = 0.000, area under the curve (AUC) 0.882), suggesting its potential capability in the differential diagnosis of erythrodermic cutaneous T-cell lymphoma from erythrodermic inflammatory dermatoses. Moreover, in contrast to erythrodermic inflammatory dermatoses, the percentage of Th17 cells was significantly downregulated in erythrodermic cutaneous T-cell lymphoma (p = 0.001), demonstrating a dysregulated immune environment in erythrodermic cutaneous T-cell lymphoma.
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- 2023
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7. Multisystemic eosinophilic epitheliotropic disease in three donkeys.
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Paraschou G, Vogel PE, Lee AM, Trawford RF, and Priestnall SL
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- Horses, Animals, Equidae, Dermatitis, Exfoliative pathology, Dermatitis, Exfoliative veterinary, Eosinophilia pathology, Eosinophilia veterinary, Horse Diseases pathology
- Abstract
Multisystemic eosinophilic epitheliotropic disease (MEED) is a rare condition of equids characterized by eosinophilic infiltration of multiple organs. Clinical signs are variable depending on the affected organs. The most common clinical signs include chronic weight loss, diarrhoea and exfoliative dermatitis. Respiratory distress and raised liver enzymes are less frequently seen. The cause is unknown and the pathogenesis is poorly understood. There are less than 50 reported cases of horses with MEED. We now document the lesions in three donkeys with fluctuating or chronic loss of weight, lethargy, exfoliative dermatitis and peripheral eosinophilia. All three animals were euthanized due to poor prognosis and welfare concerns. Post-mortem examination revealed multiple white to tan, irregular masses composed of eosinophilic infiltrates, including eosinophilic granulomas in several organs, confirming the presence of MEED. To the best of our knowledge, MEED has not previously been reported in donkeys., (Copyright © 2023 Elsevier Ltd. All rights reserved.)
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- 2023
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8. Severe Erythroderma Due to Adult-onset Still's Disease-like Disease Related to Graft-versus-host Disease after Allogeneic Hematopoietic Stem Cell Transplantation.
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Teramoto-Shibuya A, Funamizu K, Sawa N, Suenaga A, Oba Y, Ikuma D, Yamanouchi M, Hasegawa E, Sekine A, Suwabe T, Kono K, Kinowaki K, Fujii T, Takemura T, Hayashi N, Nishida A, Mori Y, Wake A, and Ubara Y
- Subjects
- Adult, Female, Humans, Skin pathology, Erythema pathology, Still's Disease, Adult-Onset complications, Still's Disease, Adult-Onset diagnosis, Still's Disease, Adult-Onset drug therapy, Dermatitis, Exfoliative etiology, Dermatitis, Exfoliative pathology, Hematopoietic Stem Cell Transplantation adverse effects
- Abstract
A 35-year-old woman was treated with chemotherapy for leukemia. One year later, allogeneic hematopoietic stem cell transplantation (HSCT) was performed with umbilical cord blood. After nine months, she developed a spiking fever, sore throat, arthralgia, pleural effusion, hyperferritinemia, and persistent generalized pruritic erythema. A skin biopsy showed dyskeratotic cells in the epidermis, neutrophil infiltration in the epidermis and upper dermis, and neutrophils in the parakeratotic layer. Treatment with tocilizumab was effective. Adult-onset Still's disease (AOSD)-like disease related to graft versus-host disease (GVHD) after HSCT was suspected. Abnormal immune states related to GVHD may cause AOSD-like disease with more severe skin lesions than usual.
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- 2023
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9. A High Programmed Cell Death Protein 1 Hormone Receptor Score on Skin Biopsy is Associated with Sézary Syndrome Diagnosis: A Study of 91 Patients with Erythroderma.
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Luherne C, Menguy S, Ferte T, Beylot-Barry M, Seneschal J, Milpied B, Vial JP, Gros A, Amintas S, Vergier B, and Pham-Ledard A
- Subjects
- Biopsy, Hormones, Humans, Programmed Cell Death 1 Receptor, Retrospective Studies, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative pathology, Drug Eruptions, Mycosis Fungoides pathology, Psoriasis, Sezary Syndrome diagnosis, Sezary Syndrome pathology, Skin Neoplasms pathology
- Abstract
Erythroderma is challenging to diagnose. The aim of this single-centre retrospective study was to identify factors that can be used to improve the diagnosis of erythroderma. Among 91 patients with erythroderma, 21 were diagnosed with eczema, 17 with psoriasis, 20 with drug-induced erythroderma, 13 with erythrodermic mycosis fungoides and 20 with Sézary syndrome. Nail alterations, ear involvement, and severe scaling were significantly associated with psoriasis (p = 0.044). Fever and hypereosinophilia were associated with drug-induced erythroderma. Expression of programmed cell death protein 1 was observed in all skin biopsies. However, with Sézary syndrome, programmed cell death protein 1 expression was significantly higher than with other aetiologies. A programmed cell death protein 1 hormone receptor score (H-score) >50 was associated with Sézary syndrome (p < 0.001, sensitivity 75%, specificity 92%) as well as CXCL13 expression (p < 0.044). CD7 loss was more frequent with erythrodermic mycosis fungoides and Sézary syndrome (p = 0.022). This study reports the importance of programmed cell death protein 1 expression for the differential diagnosis of Sézary syndrome and other aetiologies, including erythrodermic mycosis fungoides.
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- 2022
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10. A novel pathogenic variant in the corneodesmosin gene causing generalized inflammatory peeling skin syndrome with marked eosinophilia and trichorrhexis invaginata.
- Author
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Gordon H, Yap P, Hsiao KC, Watson M, and Purvis D
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- Humans, Infant, Intercellular Signaling Peptides and Proteins, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative genetics, Dermatitis, Exfoliative pathology, Eosinophilia, Hair Diseases, Netherton Syndrome diagnosis, Netherton Syndrome genetics, Skin Diseases, Genetic diagnosis, Skin Diseases, Genetic genetics
- Abstract
Generalized inflammatory peeling skin syndrome (PSS) is a rare autosomal recessive genodermatosis caused by loss-of-function disease-causing variants of the corneodesmosin gene (CDSN), resulting in excessive shedding of the superficial layers of the epidermis. We describe a case of generalized inflammatory PSS in an infant, presenting at day two of life with ichthyosiform erythroderma and superficial peeling of the skin. Hair microscopy showed trichorrhexis invaginata. Normal amounts of skin LEKT1, a product of SPINK5 on immunohistochemical staining excluded a diagnosis of Netherton syndrome. Genetic analysis revealed a homozygous novel complete CDSN deletion, estimated 4.6 kb in size, supporting the diagnosis of generalized inflammatory PSS., (© 2022 The Authors. Pediatric Dermatology published by Wiley Periodicals LLC.)
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- 2022
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11. Sézary Syndrome: Different Erythroderma Morphological Features with Proposal for a Clinical Score System.
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Roccuzzo G, Giordano S, Avallone G, Rubatto M, Canonico S, Funaro A, Ortolan E, Senetta R, Fava P, Fierro MT, Ribero S, and Quaglino P
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- Humans, Prospective Studies, Retrospective Studies, Dermatitis, Exfoliative pathology, Lymphoma, T-Cell, Cutaneous pathology, Mycosis Fungoides pathology, Sezary Syndrome pathology, Skin Neoplasms pathology
- Abstract
Sézary syndrome is a rare subtype of cutaneous T-cell lymphoma characterized by erythroderma, peripheral lymphadenopathies, and circulating atypical cerebriform T-cells. To date, no definite staging system has been developed for these patients. In this retrospective analysis of the archive of the Dermatological Clinic of the University of Turin, Italy, erythrodermic SS patients were classified according to clinical records and photographs into three main presentations: erythematous, infiltrated, or melanodermic. The pattern of erythroderma was found to be associated with disease outcome, as better survivals were recorded in patients with erythematous and infiltrative erythroderma. Patients in the melanodermic group, though less represented in our investigation, seemed to show a worse trend in survival. According to this preliminary evidence, a new prognostic classification, with a revised score specific for Sézary syndrome patients, can be proposed to usefully integrate the current staging system. The correlation displayed in our research will be hopefully confirmed by prospective studies with larger cohorts, with the aim of identifying significant prognostic features in this subset of cutaneous T-cell lymphoma patients.
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- 2022
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12. Etodolac-induced erythroderma.
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Seara V, Costa-Fernandes S, Duarte M, and Maia L
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- Aged, Anti-Infective Agents, Local therapeutic use, Anti-Inflammatory Agents therapeutic use, Betamethasone therapeutic use, Dermatitis, Exfoliative drug therapy, Dermatitis, Exfoliative pathology, Emollients, Female, Humans, Prednisolone therapeutic use, Cyclooxygenase 2 Inhibitors adverse effects, Dermatitis, Exfoliative chemically induced, Etodolac adverse effects
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- 2021
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13. Leonine facies: A unique presentation of T-prolymphocytic leukemia.
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Hasan F, Parashar Y, Rao RN, and Kashyap R
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- Antigens, CD analysis, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Cyclophosphamide therapeutic use, Dermatitis, Exfoliative diagnosis, Doxorubicin therapeutic use, Facies, Humans, Leukemia, Prolymphocytic, T-Cell drug therapy, Lymphocyte Count, Male, Middle Aged, Prednisone therapeutic use, Skin pathology, Vincristine therapeutic use, Dermatitis, Exfoliative pathology, Leukemia, Prolymphocytic, T-Cell diagnosis, Leukemia, Prolymphocytic, T-Cell pathology
- Abstract
We report a 52-year-old man who presented with erythroderma and nodular lesions on face manifesting as "Leonine facies". He had impaired sensation over the face and was initially diagnosed to have lepromatous leprosy and was treated with antileprosy drugs. Investigations showed a total Leukocyte count of 550 X 10
9 /l with 90% atypical lymphoid cells with prominent central nucleolus suggestive of prolymphocytes. On flow cytometry, these cells were positive for cytoplasmic CD3, CD2, CD5, CD7, CD4, and CD38 (dim) and were negative for CD1a and TdT and diagnosis of T-prolymphocytic leukemia was made.- Published
- 2021
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14. Interstitial granuloma after interferon-gamma and narrowband UVB therapy in a patient with mycosis fungoides: Immunological and histopathological considerations.
- Author
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Goto H, Sugita K, and Yamamoto O
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- Adult, Aged, Antiviral Agents adverse effects, Antiviral Agents therapeutic use, Biopsy methods, Combined Modality Therapy methods, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative pathology, Female, Granuloma chemically induced, Granuloma diagnosis, Histiocytes drug effects, Histiocytes pathology, Humans, Immunohistochemistry methods, Interferon-gamma therapeutic use, Male, Middle Aged, Mycosis Fungoides diagnosis, Mycosis Fungoides drug therapy, Mycosis Fungoides radiotherapy, Receptors, CXCR3 metabolism, Skin Neoplasms pathology, Th1 Cells drug effects, Th1 Cells pathology, Th1 Cells radiation effects, Ultraviolet Therapy methods, Granuloma immunology, Granuloma pathology, Interferon-gamma adverse effects, Mycosis Fungoides pathology, Ultraviolet Therapy adverse effects
- Abstract
In mycosis fungoides (MF), cutaneous granuloma formation is unusual. Furthermore, MF showing interstitial granuloma, a rare type, after combination therapy with interferon-gamma (IFN-γ) and narrowband UVB (nbUVB) has not been previously reported. A 77-year-old man was referred to our hospital with a 2-month history of erythroderma. Biopsied specimens revealed infiltration of atypical lymphocytes and eosinophils. A diagnosis of an erythrodermic variant of MF was made. He was treated with combination therapy of IFN-γ and nbUVB. After the therapy, papules newly appeared and a histopathological specimen revealed interstitial granuloma. There were several CXCR3-positive cells around the granuloma. We speculated that the combination therapy made T-helper 1 cells migrate to the cutaneous lesion and resulted in the granuloma formation. Furthermore, judging from the disappearance of elastic fibers around the interstitial granuloma, we considered that IFN-γ may induce the infiltration of histiocytes interstitially after damage of elastic fibers caused by nbUVB therapy, and both IFN-γ and nbUVB may thus play an important role in the histogenesis. Not only histopathology but also immunological observations are needed to elucidate the mechanisms underlying the development of different types of granuloma in MF., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
- Published
- 2021
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15. Deck-chair sign: unreserved.
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van Rhijn BD, van Ruth S, and Balak DMW
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- Aged, Dermatitis, Exfoliative etiology, Diagnosis, Differential, Humans, Male, Pityriasis Rubra Pilaris complications, Pruritus etiology, Dermatitis, Exfoliative pathology, Pityriasis Rubra Pilaris pathology
- Published
- 2021
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16. From SARS-CoV-2 hematogenous spreading to endothelial dysfunction: clinical-histopathological study of cutaneous signs of COVID-19.
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Patrì A, Vargas M, Buonanno P, Annunziata MC, Russo D, Staibano S, Servillo G, and Fabbrocini G
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- Aged, 80 and over, COVID-19 complications, COVID-19 virology, Dermatitis, Exfoliative drug therapy, Dermatitis, Exfoliative virology, Endothelium, Vascular drug effects, Endothelium, Vascular virology, Fatal Outcome, Host-Pathogen Interactions, Humans, Male, Parapsoriasis drug therapy, Parapsoriasis virology, Skin drug effects, Skin virology, Treatment Outcome, COVID-19 Drug Treatment, COVID-19 pathology, Dermatitis, Exfoliative pathology, Endothelium, Vascular pathology, Parapsoriasis pathology, SARS-CoV-2 pathogenicity, Skin pathology
- Abstract
Background: To date, very few studies on clinical-histopathological correlations of cutaneous disorders associated with COVID-19 have been conducted., Case Presentation: The Case 1 was a 90-year-old man, who tested positive for SARS-CoV-2 from a nasopharyngeal swab. Two days later, he was hospitalized and after eleven days transferred to Intensive Care Unit. A chest CT showed bilateral ground-glass opacities. Just that day, an erythematous maculo-papular rash appeared on trunk, shoulders and neck, becoming purpuric after few days. Histological evaluations revealed a chronic superficial dermatitis with purpuric aspects. The superficial and papillary dermis appeared edematous, with a perivascular lympho-granulocytic infiltrate and erythrocytic extravasation. At intraepithelial level, spongiosis and a granulocyte infiltrate were detected. Arterioles, capillaries and post-capillary venules showed endothelial swelling and appeared ectatic. The patient was treated with hydroxychloroquine, azithromycin, lopinavir-ritonavir and tocilizumab. Regrettably, due to severe lung impairment, he died. The Case 2 was a 85-year-old man, admitted to Intensive Care Unit, where he was intubated. He had tested positive for SARS-CoV-2 from a nasopharyngeal swab two days before. A chest RX showed bilateral atypical pneumonia. After seven days, a cutaneous reddening involving trunk, upper limbs, neck and face developed, configuring a sub-erythroderma. Histological evaluations displayed edema in the papillary and superficial reticular dermis, and a perivascular lymphocytic infiltrate in the superficial dermis. The patient was treated with hydroxychloroquine, azithromycin, lopinavir-ritonavir and tocilizumab. Sub-erythroderma as well as respiratory symptoms gradually improved until healing., Conclusions: The endothelial swelling detected in the Case 1 could be a morphological expression of SARS-CoV-2-induced endothelial dysfunction. We hypothesize that cutaneous damage could be initiated by endothelial dysfunction, caused by SARS-CoV-2 infection of endothelial cells or induced by immune system activation. The disruption of endothelial integrity could enhance microvascular permeability, extravasation of inflammatory cells and cytokines, with cutaneous injury. The Case 2 developed a sub-erythroderma associated with COVID-19, and a non-specific chronic dermatitis was detected at histological level. We speculate that a purpuric rash could represent the cutaneous sign of a more severe coagulopathy, as highlighted histologically by vascular abnormalities, while a sub-erythroderma could be expression of viral hematogenous spreading, inducing a non-specific chronic dermatitis.
- Published
- 2021
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17. Neonatal erythroderma.
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Cotter CL, Rivers E, Salisbury J, Duarte Williamson E, and Tewari A
- Subjects
- Biopsy, Dermatitis, Exfoliative pathology, Diagnosis, Differential, Humans, Infant, Newborn, Male, Severe Combined Immunodeficiency complications, Severe Combined Immunodeficiency pathology, Dermatitis, Exfoliative etiology, Severe Combined Immunodeficiency diagnosis, Skin pathology
- Published
- 2020
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18. Erythroderma: a prospective study of 309 patients followed for 12 years in a tertiary center.
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Miyashiro D and Sanches JA
- Subjects
- Dermatitis, Exfoliative classification, Female, Follow-Up Studies, Humans, Male, Middle Aged, Prognosis, Prospective Studies, Time Factors, Dermatitis, Exfoliative etiology, Dermatitis, Exfoliative pathology, Skin Diseases complications, Tertiary Care Centers statistics & numerical data
- Abstract
Erythroderma is characterized by erythema and scaling affecting more than 80% of the body surface area. It is potentially life-threatening, and diagnosis of the underlying disease is a challenge. Despite laboratory improvements, many cases remain idiopathic. We aimed to analyze clinical and laboratory findings of 309 erythrodermic patients to find clues to the etiologic diagnosis. We performed a prospective study at the University of São Paulo Medical School, from 2007 to 2018, with patients with acquired erythroderma. Clinical, laboratory, histology, and molecular biology data were collected. The median age at diagnosis was 57 years, with a male-to-female ratio of 2.2. Eczema was the most frequent etiology (20.7%), followed by psoriasis (16.8%), Sézary syndrome (12.3%), drug eruption (12.3%), atopic dermatitis (8.7%), and mycosis fungoides (5.5%). Other diagnoses (6.8%) included pemphigus foliaceous, paraneoplastic erythroderma, adult T-cell leukemia/lymphoma, dermatomyositis, pityriasis rubra pilaris, lichen planus, bullous pemphigoid, and leprosy. In 52 patients (16.8%), it was not possible to elucidate erythroderma etiology. Atopic dermatitis developed erythroderma at an earlier age (median 25 years; P = 0.0001). Acute onset was associated with drug reactions and atopic dermatitis (median time from erythroderma to diagnosis of 1 and 1.5 months, respectively; P = 0.0001). Higher immunoglobulin E levels were observed in atopic dermatitis (median 24,600 U/L; P = 0.0001). Histopathology was helpful and was consistent with the final diagnosis in 72.4%. Monoclonal T-cell proliferation in the skin was observed in mycosis fungoides (33.3%) and Sézary syndrome (90.9%). At the last assessment, 211 patients (69.3%) were alive with disease, 65 (21.7%) were alive without disease, and 27 (9.1%) died with active disease. Erythroderma is a challenging syndrome with a difficult diagnostic approach. Younger age and higher immunoglobulin E levels are associated with atopic dermatitis; acute onset is observed in drug eruptions and atopic dermatitis. Histopathology and molecular biology tests are essential tools in the investigation of erythroderma.
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- 2020
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19. Case of erythrodermic lichen planus successful treated with thalidomide monotherapy.
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Chen X and Wang S
- Subjects
- Administration, Oral, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative etiology, Dermatitis, Exfoliative pathology, Humans, Lichen Planus complications, Lichen Planus diagnosis, Lichen Planus pathology, Male, Middle Aged, Skin pathology, Treatment Outcome, Dermatitis, Exfoliative drug therapy, Lichen Planus drug therapy, Thalidomide administration & dosage
- Published
- 2020
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20. Erythroderma as a paraneoplastic manifestation of small cell lung cancer.
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Zervakis SG, Spernovasilis N, Boutakoglou E, Panagiotakis S, Thomopoulou K, and Samonis G
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- Aged, Dermatitis, Exfoliative etiology, Humans, Male, Prognosis, Dermatitis, Exfoliative pathology, Lung Neoplasms complications, Small Cell Lung Carcinoma complications
- Abstract
Erythroderma is a rare clinical entity characterized by generalized erythema affecting the whole or most of the body's skin surface. It is associated with a variety of underlying conditions, including preexisting dermatoses, infections, connective tissue disorders, drugs, malignancies, or it may be idiopathic. A case of a 73-year-old man, with a 5-month history of erythroderma, eventually diagnosed with small cell lung cancer is presented. This is the first reported case indicating an association between erythroderma and small cell lung cancer, extending, thus, current knowledge regarding small cell lung cancer-related paraneoplastic manifestations as well as erythroderma's causative factors., (Copyright © 2019 Elsevier Inc. All rights reserved.)
- Published
- 2020
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21. Methotrexate for papuloerythroderma of Ofuji.
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Qureshi F, Hughes AJ, and Natkunarajah J
- Subjects
- Aged, 80 and over, Dermatitis, Exfoliative pathology, Eosinophilia complications, Folliculitis complications, Humans, Male, Pruritus drug therapy, Pruritus etiology, Skin Diseases, Vesiculobullous complications, Dermatologic Agents therapeutic use, Eosinophilia drug therapy, Folliculitis drug therapy, Methotrexate therapeutic use, Skin pathology, Skin Diseases, Vesiculobullous drug therapy
- Published
- 2020
- Full Text
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22. Painless erosions on the hands and feet of a 7-year-old boy.
- Author
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Schimmel J, Renzi M, Fawaz B, and Halpern A
- Subjects
- Child, Dermatitis, Exfoliative complications, Dermatitis, Exfoliative genetics, Dermatitis, Exfoliative pathology, Extremities, Humans, Male, Pain etiology, Skin Diseases, Genetic complications, Skin Diseases, Genetic genetics, Skin Diseases, Genetic pathology, Dermatitis, Exfoliative diagnosis, Skin pathology, Skin Diseases, Genetic diagnosis
- Published
- 2020
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23. [Ichtyosiform erythroderma revealing a severe combined immunodeficiency].
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Ghariani Fetoui N, Boussofara L, Hmida D, Mokni S, Mekki N, Ben Mustapha I, Belajouza C, Ghariani N, Picard C, and Denguezli M
- Subjects
- Alopecia etiology, Alopecia pathology, Chronic Disease, Consanguinity, DNA-Binding Proteins genetics, Dermatitis, Exfoliative pathology, Diarrhea etiology, Eczema etiology, Eczema pathology, Hematopoietic Stem Cell Transplantation, Humans, Infant, Male, Nuclear Proteins genetics, Photography, Severe Combined Immunodeficiency genetics, Severe Combined Immunodeficiency therapy, Dermatitis, Exfoliative etiology, Severe Combined Immunodeficiency complications
- Abstract
Background: Severe combined immunodeficiency (SCID) is a the most severe form of primary immunodeficiency and is highly heterogeneous. We report an atypical form of SCID revealed by exfoliative erythroderma., Patients and Methods: A 3-month-old boy, born to consanguineous parents, was admitted to the dermatology department with exfoliative erythroderma associated with eczematous patches and alopecia of the scalp, eyelashes, and eyebrows, but with no lymphadenopathy or hepatosplenomegaly. He displayed chronic diarrhea and recurrent infection since birth. A complete blood count showed marked leukocytosis with eosinophilia and lymphocytosis. These clinical and biological findings improved partly with topical steroids. The patient no longer had erythroderma and showed regrowth of hair, eyelashes and eyebrows. The subsequent CBC showed less marked eosinophilia with mild lymphopenia and no leukocytosis. Immunoglobulin levels were undetectable. Primary immunodeficiency was discussed. Immunological investigations concluded on a diagnosis of T-B-NK+ SCID. Mutation analysis revealed a homozygous c.1338C>G (pCys446Trp) mutation in the RAG2 gene. Hematopoietic stem cell transplantation is planned in the near future., Conclusion: This case illustrates atypical T-B-NK+ SCID revealed by severe exfoliative erythroderma in a 3-month-old boy with RAG2 gene mutation. Neonatal erythroderma must be considered a warning sign of primary immunodeficiency requiring immediate immunological phenotyping as well as genetic testing for a definitive diagnosis., (Copyright © 2019. Published by Elsevier Masson SAS.)
- Published
- 2020
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24. Keratinocytes apoptosis contributes to crizotinib induced-erythroderma.
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Hu Y, Zhang X, Zhao Z, Chen X, Zhou Z, Yang X, Yang B, He Q, and Luo P
- Subjects
- Amino Acid Chloromethyl Ketones pharmacology, Caspase 8 metabolism, Caspase Inhibitors pharmacology, Cell Line, DNA Damage, Dermatitis, Exfoliative pathology, Humans, Membrane Potential, Mitochondrial drug effects, Primary Cell Culture, Reactive Oxygen Species metabolism, Signal Transduction drug effects, Antineoplastic Agents toxicity, Apoptosis drug effects, Crizotinib toxicity, Dermatitis, Exfoliative chemically induced, Keratinocytes drug effects
- Abstract
Crizotinib is a multi-target receptor tyrosine kinase inhibitor which is of great importance for the management of ALK-rearranged non-small cell lung cancer (NSCLC) patients. Serious erythroderma and toxic epidermal necrolysis have been reported associated with crizotinib treatment. The underlying mechanisms have not been examined. In this study, we tested the toxicity of crizotinib on immortal human keratinocytes (HaCaT) and human primary keratinocytes. We found that crizotinib directly cause cytotoxic on these two cells, which could be the explanation of the clinical characteristic of pathology. Apoptosis was observed and Z-VAD-FMK, a pan-caspase inhibitor can almost totally reverse the apoptosis induction effect of crizotinib. However, mitochondrial dysfunction and DNA damage were not involved in crizotinib-induced apoptosis, indicating the intrinsic apoptosis pathway have no connection with this cutaneous toxicity. Further studies showed that crizotinib significantly increased cleaved-caspase-8, a signaling protein of extrinsic apoptosis pathway, in a concentration and time-dependent manner. Moreover, we found the targets of crizotinib were not involved in HaCaT cells apoptosis. Collectively, our findings first report keratinocytes apoptosis is the key cause of crizotinib-induced cutaneous toxicity. We also reveal crizotinib induce apoptosis through the extrinsic apoptosis pathway due to detected up-regulated cleaved-caspase-8. Meanwhile, the apoptosis is independent of mitochondrial dysfunction, DNA damage and related drug targets inhibition., (Copyright © 2019 Elsevier B.V. All rights reserved.)
- Published
- 2020
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25. Familial "benign" pemphigus? Erythroderma and fatal outcome.
- Author
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Tansini PB, Boff AL, Weber MB, and Bonamigo RR
- Subjects
- Acantholysis pathology, Catheter-Related Infections, Dermatitis, Exfoliative complications, Dermatitis, Exfoliative drug therapy, Fatal Outcome, Female, Humans, Middle Aged, Pemphigus, Benign Familial complications, Pemphigus, Benign Familial drug therapy, Dermatitis, Exfoliative pathology, Pemphigus, Benign Familial pathology
- Abstract
Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome., (Copyright © 2020 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U. All rights reserved.)
- Published
- 2020
- Full Text
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26. Erythroderma as first manifestation of squamous cell lung cancer: rare case report.
- Author
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Arandes-Marcocci J, Iglesias-Sancho M, Setó-Torrent N, and Fernández-Figueras MT
- Subjects
- Aged, Biopsy, Dermatitis, Exfoliative etiology, Erythema pathology, Humans, Lung Neoplasms complications, Male, Neoplasms, Squamous Cell complications, Paraneoplastic Syndromes pathology, Tomography, X-Ray Computed, Dermatitis, Exfoliative pathology, Lung Neoplasms pathology, Neoplasms, Squamous Cell pathology
- Abstract
Erythroderma as the first manifestation of a solid organ malignancy is rare. The underlying cancer is a challenging condition to diagnose. There are a few cases of erythroderma in cancer patients reported in the literature. We here describe the case of a 70-year-old man who presented with asthenia, weight loss, dry cough and total body erythema with desquamation over the past month. A chest computed tomography scan showed a nodular lesion, which was finally diagnosed as a squamous cell lung carcinoma. To our knowledge, as an erythroderma presentation, only 13 cases have been reported in the literature. This case report demonstrates the need to search for a neoplasm in patients presenting with erythroderma, particularly in the presence of accompanying debilitating symptoms., (Copyright © 2019 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U. All rights reserved.)
- Published
- 2020
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- View/download PDF
27. Generalized Skin Scaling in a Young Girl: A Quiz.
- Author
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Mo R, Wang H, and Lin Z
- Subjects
- Adolescent, Biopsy, Needle, Dermatitis, Exfoliative diagnosis, Female, Filaggrin Proteins, Humans, Immunohistochemistry, Monitoring, Physiologic, Prognosis, Rare Diseases, Skin Diseases, Genetic diagnosis, Dermatitis, Exfoliative genetics, Dermatitis, Exfoliative pathology, Genetic Predisposition to Disease, S100 Proteins genetics, Skin Abnormalities genetics, Skin Abnormalities pathology, Skin Diseases, Genetic genetics, Skin Diseases, Genetic pathology
- Published
- 2019
- Full Text
- View/download PDF
28. Thymoma-associated exfoliative dermatitis in a goat: case report and brief literature review.
- Author
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Byas AD, Applegate TJ, Stuart A, Byers S, and Frank CB
- Subjects
- Animals, Colorado, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative pathology, Fatal Outcome, Female, Goat Diseases pathology, Goats, Skin pathology, Thymoma diagnosis, Thymoma pathology, Thymus Neoplasms diagnosis, Thymus Neoplasms pathology, Dermatitis, Exfoliative veterinary, Goat Diseases diagnosis, Thymoma veterinary, Thymus Neoplasms veterinary
- Abstract
A Rock Alpine doe ( Capra aegagrus hircus ) was presented to the Colorado State University Veterinary Teaching Hospital because of scaling and ulceration over the withers, coronary bands, and dewclaws. The doe was euthanized because of poor prognosis associated with a radiographically identified cranial mediastinal mass, increased respiratory effort, and discomfort. Autopsy revealed a cranial mediastinal mass, and scaling-to-ulcerative lesions affecting the dorsum, ventrum, pinna, neck, teats, coronary bands, and dewclaws. Histologically, the mediastinal mass was an epithelial neoplasm with admixed non-neoplastic T lymphocytes, consistent with a lymphoepithelial (mixed) thymoma. Sections of affected skin were characterized by hyperkeratotic cell-rich interface dermatitis with transepidermal and follicular apoptosis. Thymoma-associated exfoliative dermatitis has been recognized in cats and a rabbit, but has not been reported previously in a goat, to our knowledge. Given that thymomas are not uncommon in goats, thymoma-associated exfoliative dermatitis should be considered a clinical differential in goats with dermatologic disease.
- Published
- 2019
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- View/download PDF
29. Adalimumab-induced erythrodermic reactions.
- Author
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Nowsheen S, Kohorst JJ, and El-Azhary RA
- Subjects
- Aged, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative pathology, Female, Humans, Male, Middle Aged, Skin drug effects, Skin pathology, Young Adult, Adalimumab adverse effects, Anti-Inflammatory Agents adverse effects, Dermatitis, Exfoliative chemically induced, Psoriasis drug therapy
- Published
- 2019
- Full Text
- View/download PDF
30. Norwegian scabies presenting as erythroderma in HIV: A case report.
- Author
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Paparizos V, Vasalou V, Velissariou E, Kourkounti S, Daskalakis E, and Rigopoulos D
- Subjects
- Dermatitis, Exfoliative drug therapy, Dermatitis, Exfoliative etiology, Dermatitis, Exfoliative pathology, HIV Infections drug therapy, Humans, Male, Middle Aged, Scabies pathology, Substance-Related Disorders complications, HIV Infections complications, Scabies drug therapy
- Abstract
Crusted scabies is a rare and extreme manifestation of scabies that is observed mainly among immunosuppressed patients. We describe the case of a 55-year-old patient with a history of HIV infection and injection drug use. The patient was not on any antiretroviral therapy during the previous year, was malnourished and was living in unsanitary conditions. He had extensive, generalized, thick, hyperkeratotic, crusting, papular lesions, which had evolved over the previous month. Hyperkeratotic areas were fissured and linear excoriations were noted diffusely. The rash was distributed on the entire body from the scalp to the toes, with mild itching. Microscopic examination of the scale revealed numerous scabies mites and eggs. The patient was treated with topical scabicidal agents, which resulted in complete resolution. Because of the extremely contagious nature of crusted scabies, as well as its potential for complete cure with an appropriate therapy, there should be a high degree of suspicion for this disease in patients with AIDS, even when the lesions do not have the classical appearance. Nosocomial transmission of scabies from patients with AIDS is a risk, and protective measures, early diagnosis, and therapy are essential.
- Published
- 2019
31. Therapeutic options and prognosis of chronic idiopathic erythroderma in older adults.
- Author
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Ohga Y, Bayaraa B, and Imafuku S
- Subjects
- Administration, Oral, Aged, Aged, 80 and over, Chronic Disease, Dermatitis, Exfoliative pathology, Dermatologic Agents administration & dosage, Female, Humans, Male, Prognosis, Recurrence, Retrospective Studies, Treatment Outcome, Adrenal Cortex Hormones administration & dosage, Cyclosporine administration & dosage, Dermatitis, Exfoliative therapy, Ultraviolet Therapy methods
- Abstract
The present authors have previously reported that chronic idiopathic erythroderma (CIE) that lasts for more than 3 months without specific causes occurs almost exclusively in older men. No standardized therapeutic treatments for the condition have been established to date. To assess the efficacies and safeties of current treatments for CIE, the present authors reviewed cases of CIE. Twenty cases were identified, of which CIE was treated with oral corticosteroids, oral cyclosporine A (CsA), ultraviolet therapy, or a combination of these therapies. CIE was cured in 8/20 (40%) of these cases, and the average time to cure was 19.3 months. Oral CsA was the most effective treatment at relatively low doses (50-100 mg/day), which resulted in cures in four cases. Ultraviolet therapy also effectively cured two subjects, and oral corticosteroids cured one patient. In patients that were treated with oral CsA or corticosteroids, the terminations of the treatments within 2 to 3 months resulted in relapses in multiple patients. The maintaining of the treatments for a certain period may be necessary in cases that involve treatments with these orally administered drugs. Although CIE is a chronic and potentially refractory disease, it can evidently be cured or well controlled via appropriate long-term therapies., (© 2019 Wiley Periodicals, Inc.)
- Published
- 2019
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32. Progressive Erythroderma without Malignancy in the Skin: A Quiz.
- Author
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Irie H, Dainichi T, Kaku Y, and Kabashima K
- Subjects
- Dermatitis, Exfoliative pathology, Disease Progression, Female, Humans, Middle Aged, Skin pathology, Thymectomy, Thymoma immunology, Thymoma pathology, Thymoma surgery, Thymus Neoplasms immunology, Thymus Neoplasms pathology, Thymus Neoplasms surgery, Treatment Outcome, Tumor Burden, Autoimmunity, Dermatitis, Exfoliative immunology, Skin immunology, Thymoma complications, Thymus Neoplasms complications
- Published
- 2019
- Full Text
- View/download PDF
33. PLACK syndrome shows remarkable phenotypic homogeneity.
- Author
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Mohamad J, Samuelov L, Ben-Amitai D, Malchin N, Sarig O, and Sprecher E
- Subjects
- Calcium-Binding Proteins genetics, Cheilitis genetics, Cheilitis pathology, Dermatitis, Exfoliative genetics, Dermatitis, Exfoliative pathology, Humans, Keratosis genetics, Keratosis pathology, Male, Middle Aged, Mutagenesis, Insertional genetics, Pedigree, Skin pathology, Skin Diseases, Genetic genetics, Skin Diseases, Genetic pathology, Syndrome, Cheilitis diagnosis, Dermatitis, Exfoliative diagnosis, Keratosis diagnosis, Skin Diseases, Genetic diagnosis
- Published
- 2019
- Full Text
- View/download PDF
34. Severe dermatitis, multiple allergies and metabolic wasting (SAM) syndrome caused by de novo mutation in the DSP gene misdiagnosed as generalized pustular psoriasis and treatment of acitretin with gabapentin.
- Author
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Liang J, Li C, Zhang Z, Ni C, Yu H, Li M, and Yao Z
- Subjects
- Administration, Cutaneous, Administration, Oral, Child, Preschool, DNA Mutational Analysis, Dermatitis, Exfoliative drug therapy, Dermatitis, Exfoliative genetics, Dermatitis, Exfoliative pathology, Diagnosis, Differential, Diagnostic Errors, Female, Humans, Hypersensitivity drug therapy, Hypersensitivity genetics, Hypersensitivity pathology, Mutation, Missense, Psoriasis diagnosis, Severity of Illness Index, Skin drug effects, Skin pathology, Syndrome, Tacrolimus administration & dosage, Tacrolimus analogs & derivatives, Treatment Outcome, Wasting Syndrome drug therapy, Wasting Syndrome genetics, Wasting Syndrome pathology, Acitretin administration & dosage, Dermatitis, Exfoliative diagnosis, Desmoplakins genetics, Gabapentin administration & dosage, Hypersensitivity diagnosis, Wasting Syndrome diagnosis
- Abstract
Severe dermatitis, multiple allergies and metabolic wasting (SAM) syndrome is a recently recognized syndrome caused by mutations in the desmoglein 1 (DSG1) and desmoplakin (DSP) genes. Only two cases of SAM-DSP have been reported. We report on a 2-year-old girl presenting with pustular lakes within areas of erythema and large accumulations of intraepidermal neutrophils, which initially led to our misdiagnosis of generalized pustular psoriasis. No mutation was found in either the IL36RN or CARD14 genes by Sanger sequencing. The distinctive manifestations of erythroderma with severe itching, hypotrichosis, enamel defects, onychodystrophy, palmoplantar keratoderma and the crucial result of de novo missense mutation in exon 14 of the DSP gene (c.1828T>C, p.S610P) discovered by next-generation sequencing finally confirmed the diagnosis of SAM syndrome. The eruptions significantly improved after a 4-week treatment with oral acitretin and topical pimecrolimus. Oral gabapentin was prescribed simultaneously for 4 months, relieving her skin pruritus and suggesting that early treatment with pimecrolimus, acitretin and gabapentin for SAM-DSP syndrome is effective. It may even inhibit multiple allergies induced by skin barrier injury. In this work we also review the clinical features, differential diagnoses and pathological manifestations of SAM-DSP syndrome., (© 2019 Japanese Dermatological Association.)
- Published
- 2019
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35. Case of psoriatic erythroderma induced by the discontinuation of the chronic use of topical steroid after dialysis initiation and successfully treated with secukinumab.
- Author
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Shibata T, Muto J, Takama H, Yanagishita T, Ito T, and Watanabe D
- Subjects
- Administration, Cutaneous, Adrenal Insufficiency diagnosis, Antibodies, Monoclonal, Humanized, Biopsy, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative pathology, Disease Progression, Female, Humans, Middle Aged, Psoriasis diagnosis, Psoriasis pathology, Renal Dialysis, Severity of Illness Index, Skin drug effects, Skin pathology, Time Factors, Treatment Outcome, Adrenal Insufficiency etiology, Antibodies, Monoclonal therapeutic use, Dermatitis, Exfoliative therapy, Glucocorticoids adverse effects, Psoriasis therapy
- Published
- 2019
- Full Text
- View/download PDF
36. Prognostic Significance of Serum Copper in Patients With Cutaneous T-cell Lymphoma.
- Author
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Vonderheid EC and Martinez AR
- Subjects
- Dermatitis, Exfoliative blood, Dermatitis, Exfoliative mortality, Dermatitis, Exfoliative pathology, Disease Progression, Female, Humans, Lymphoma, T-Cell, Cutaneous mortality, Lymphoma, T-Cell, Cutaneous pathology, Male, Middle Aged, Mycosis Fungoides blood, Mycosis Fungoides mortality, Mycosis Fungoides pathology, Prognosis, Sezary Syndrome blood, Sezary Syndrome mortality, Sezary Syndrome pathology, Skin Neoplasms mortality, Skin Neoplasms pathology, Survival Analysis, Copper blood, Lymphoma, T-Cell, Cutaneous blood, Skin Neoplasms blood
- Abstract
Background: Serum copper has been reported to be increased in various cancers, including lymphoma. The purpose of the present study was to investigate the clinical and prognostic importance of serum copper levels in patients with cutaneous T-cell lymphoma (CTCL)., Patients and Methods: Serum copper was measured in 60 men and 38 women with mycosis fungoides (MF) and 14 men and 3 women with erythrodermic CTCL (6 with Sézary syndrome) consecutively evaluated from July 1980 to June 1985., Results: A greater than normal copper level was present in nearly 20% of patients and was associated with an increased risk of disease progression and shortened disease-specific survival for patients with patch or plaque phase MF, but not for those with tumor phase MF or erythrodermic CTCL. In contrast, the serum lactate dehydrogenase level and neutrophil/lymphocyte ratio were not significantly associated with prognosis in our patient cohort., Conclusion: The reason for the association between the high serum copper levels and adverse prognosis is unknown. We hypothesized that interleukin-6 is secreted primarily by non-neoplastic cells at MF skin sites, leading to release of copper by the liver. Local production of interleukin-6 at the lesion sites might conceivably also promote neoplastic cell progression by stimulation of the STAT3 pathway. Further studies on the relationship between activated tumor-associated macrophages, serum copper levels, interleukin-6, or C-reactive protein and prognosis might be informative., (Copyright © 2018 Elsevier Inc. All rights reserved.)
- Published
- 2019
- Full Text
- View/download PDF
37. A case of erythrodermic psoriasis successfully treated with ixekizumab.
- Author
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Megna M, Gallo L, Balato N, and Balato A
- Subjects
- Aged, Dermatitis, Exfoliative pathology, Female, Humans, Interleukin-17 immunology, Psoriasis pathology, Treatment Outcome, Antibodies, Monoclonal, Humanized therapeutic use, Dermatitis, Exfoliative drug therapy, Dermatologic Agents therapeutic use, Psoriasis drug therapy
- Abstract
Erythrodermic psoriasis (EP) is the most severe form of psoriasis, resulting in significant morbidity and mortality. International guidelines on EP treatment are lacking, with most of the biologic drugs being used basing on case reports or small case series. Ixekizumab, a fully human anti-interleukin (IL)-17A monoclonal antibody, is approved for moderate to severe plaque psoriasis while its use in EP is off label. However, two studies conducted on eight Japanese EP patients have showed ixekizumab as an efficacious and well tolerated therapy up to 24 and 52 weeks, respectively. To date, no case reports on Caucasian patients have been described. We report the case of a 66-year-old Caucasian female with EP successfully treated with ixekizumab, reaching PASI 100 after only 6 weeks of therapy and still maintaining this response at week 24. Our case report suggests ixekizumab as a highly efficacious treatment in EP, presenting also a very rapid action which leads to complete resolution of the disease after 6 weeks. Further studies are warrant to confirm our data, with controlled trials specifically dedicated to EP being strictly needed in order to verify the role and efficacy of the new biologics in EP., (© 2019 Wiley Periodicals, Inc.)
- Published
- 2019
- Full Text
- View/download PDF
38. The rash that becomes an erythroderma.
- Author
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Inamadar AC and Ragunatha S
- Subjects
- Age of Onset, Diagnosis, Differential, Exanthema etiology, Humans, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative epidemiology, Dermatitis, Exfoliative etiology, Dermatitis, Exfoliative pathology, Exanthema pathology, Skin pathology
- Abstract
Erythroderma is a dermatologic emergency with potentially serious consequences. Several diseases with different etiologies characteristically appear as erythroderma. Depending on the age groups, congenital ichthyosiform disorders, infections, preexisting dermatoses, drug eruptions, and internal malignancies commonly present with, or progress to, erythroderma. The course, prognosis, and management strategies also vary depending on the cause of erythroderma; hence, an accurate diagnosis is essential in minimizing associated morbidity and mortality. The generalized erythema and scaling often obscure the classic clinical features of the underlying skin diseases, posing a diagnostic challenge to dermatologists. Awareness and elicitation of subtle signs and clinical manifestations are crucial. A step-wise approach ensures completeness of clinical evaluation and avoids missing any relevant clinical data. The initial clinical presentation, cutaneous examination findings, and systemic clues reveal important information regarding the diagnosis, course, and prognosis of erythroderma. The age at onset, symptomatology, duration of illness, initial lesions, initial site of onset, clinical course, family history, types of scales, changes in cutaneous integuments and systemic clues will assist in delineating the nature of underlying disease., (Copyright © 2018 Elsevier Inc. All rights reserved.)
- Published
- 2019
- Full Text
- View/download PDF
39. Dermoscopy of papuloerythroderma of Ofuji.
- Author
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Apalla Z, Koukouthaki A, Sidiropoulos T, Psarakis E, Nikolaidou C, Bobos M, Kampouropoulou E, and Lallas A
- Subjects
- Aged, Humans, Male, Dermatitis, Exfoliative pathology, Dermoscopy, Skin pathology
- Published
- 2019
- Full Text
- View/download PDF
40. Case of pityriasis rubra pilaris progressed to generalized erythroderma following blockade of interleukin-17A, but improved after blockade of interleukin-12/23 p40.
- Author
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Matsuda T, Yamazaki F, Ueda-Hayakawa I, Kambe N, and Okamoto H
- Subjects
- Aged, Dermatitis, Exfoliative immunology, Dermatitis, Exfoliative pathology, Dermatologic Agents pharmacology, Disease Progression, Humans, Male, Pityriasis Rubra Pilaris immunology, Pityriasis Rubra Pilaris pathology, Skin immunology, Skin pathology, Treatment Outcome, Dermatitis, Exfoliative drug therapy, Dermatologic Agents therapeutic use, Interleukin-12 Subunit p40 antagonists & inhibitors, Interleukin-17 antagonists & inhibitors, Pityriasis Rubra Pilaris drug therapy
- Abstract
We report herein a case of a 72-year-old man with pityriasis rubra pilaris (PRP) that was refractory to conventional therapies. His skin lesions progressed to generalized erythroderma despite anti-interleukin (IL)-17A antibody therapy. Topical corticosteroids, emollients, systemic retinoid, methotrexate, cyclosporin and phototherapy yielded no therapeutic response. However, blockade of IL-12/23 p40 dramatically improved his cutaneous lesions. Complete remission was achieved 4 weeks after the first injection of ustekinumab and maintained for more than 48 weeks. Our data indicate that IL-12 was associated with the onset of PRP in this patient, rather than IL-23. IL-12 is critical for the differentiation of T-helper (Th)1 cells. Thus, the Th1 pathway may be associated with the onset of PRP., (© 2018 Japanese Dermatological Association.)
- Published
- 2019
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- View/download PDF
41. Papuloerythroderma-like cutaneous involvement of a CD62L - subclone of T-cell prolymphocytic leukemia.
- Author
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Nakagawa Y, Hamada T, Matsuda M, Kanno T, Kondo T, Takahashi T, Watanabe T, Okada K, Kawakami T, Miyake T, Morizane S, and Iwatsuki K
- Subjects
- Aged, 80 and over, Biopsy, CD4-Positive T-Lymphocytes metabolism, Dermatitis, Exfoliative blood, Dermatitis, Exfoliative diagnosis, Fatal Outcome, Flow Cytometry, Humans, Leukemia, Prolymphocytic, T-Cell blood, Leukemia, Prolymphocytic, T-Cell diagnosis, Male, Serologic Tests, Skin pathology, Skin Neoplasms blood, Skin Neoplasms diagnosis, Dermatitis, Exfoliative pathology, L-Selectin metabolism, Leukemia, Prolymphocytic, T-Cell pathology, Skin Neoplasms pathology
- Abstract
We report the case of an 88-year-old Japanese man with erythrodermic involvement of T-cell prolymphocytic leukemia (T-PLL). He had a history of pharyngeal diffuse large B-cell lymphoma successfully treated with polychemotherapy including cyclophosphamide and epirubicin, 6 years before the current illness. He presented with numerous reddish, coalescing, flat-topped papules on the trunk and extremities, sparing the skin folds of the abdomen, the features of which mimicked those of papuloerythroderma. Immunohistochemistry showed perivascular and epidermotropic infiltration of CD3
+ CD4+ T cells in the cutaneous lesion. However, flow cytometric analysis revealed that the skin infiltrating T cells were negative for surface CD4, and that CD3+ CD4- CD8- cells made up 92% of the T-cell fraction of peripheral blood. The circulating atypical T cells had a round or oval nucleus and prominent nucleoli, and the deletion of chromosomes 6q, 13 and 17. These cytological profiles were consistent with those of T-PLL and distinct from those of Sézary cells. The same T-cell clone was detected in the cutaneous lesion and peripheral blood, but the expression of CD62L was absent in the skin infiltrates and present in the circulating cells. No specific mutation was detected in STAT3 or STAT5B. Although low-dose oral etoposide had a beneficial effect on the skin rash, a fatal crisis of marked leukocytosis (169 × 103 /μL) occurred 19 months after the illness onset. CD62L-leukemic cells of T-PLL may infiltrate the skin to form papuloerythroderma-like cutaneous lesions., (© 2018 Japanese Dermatological Association.)- Published
- 2019
- Full Text
- View/download PDF
42. Erythroderma as a manifestation of leprosy.
- Author
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Baldissera RL, Shwetz GA, Fillus Neto J, and Vigo NDR
- Subjects
- Aged, Biopsy, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative pathology, Diagnosis, Differential, Humans, Leprosy, Borderline diagnosis, Leprosy, Borderline pathology, Leprosy, Lepromatous diagnosis, Leprosy, Lepromatous pathology, Male, Dermatitis, Exfoliative complications, Leprosy, Borderline etiology, Leprosy, Lepromatous etiology
- Abstract
Erythroderma consists of erythema and scaling involving most or all of the body surface. This generalized eruption may be idiopathic, drug-induced or secondary to cutaneous or systemic disease. A 71-year-old man is reported presenting generalized erythema and desquamation with deck-chair sign, nail dystrophy, and plantar ulcers associated with loss of local tactile sensitivity. Biopsies from three different sites demonstrated diffuse lymphocytic infiltrate with incipient granulomas. Fite-Faraco staining showed numerous isolated bacilli and globi. The skin smear was positive. Clinical and pathological diagnosis of borderline lepromatous leprosy was confirmed. This report demonstrates that chronic multibacillary leprosy can manifest as erythroderma and thus should be included in the differential diagnosis.
- Published
- 2019
- Full Text
- View/download PDF
43. Langerhans Cell Histiocytosis with Lymph Node Involvement Presenting as Erythroderma.
- Author
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Zhu H, Ma Y, Sun L, Zhang R, Lv L, and Wang A
- Subjects
- Aged, Biopsy, Dermatitis, Exfoliative drug therapy, Dermatitis, Exfoliative pathology, Emollients therapeutic use, Glucocorticoids therapeutic use, Histiocytosis, Langerhans-Cell drug therapy, Histiocytosis, Langerhans-Cell pathology, Humans, Immunohistochemistry, Lymph Nodes drug effects, Male, Remission Induction, Skin drug effects, Time Factors, Treatment Outcome, Dermatitis, Exfoliative etiology, Histiocytosis, Langerhans-Cell complications, Lymph Nodes pathology, Skin pathology
- Published
- 2019
- Full Text
- View/download PDF
44. Pityriasis Rubra Pilaris With Extensive Follicular Acantholysis Resembling Pemphigus Vulgaris: A Case Report.
- Author
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Lilo MT, Yan S, Chapman MS, and Linos K
- Subjects
- Acantholysis drug therapy, Biopsy, Dermatitis, Exfoliative pathology, Diagnosis, Differential, Humans, Immunosuppressive Agents therapeutic use, Middle Aged, Pityriasis Rubra Pilaris drug therapy, Predictive Value of Tests, Skin drug effects, Steroids therapeutic use, Acantholysis pathology, Pemphigus pathology, Pityriasis Rubra Pilaris pathology, Skin pathology
- Abstract
Pityriasis rubra pilaris (PRP) is a rare, chronic, heterogeneous, papulosquamous inflammatory dermatosis of unknown etiology. Although erythematous scaly patches characterize the classic presentation of PRP, a broad range of clinical presentations has been reported. Histologically, PRP is characterized by psoriasiform acanthosis with alternating orthokeratosis and parakeratosis and rarely small acantholytic foci. In this article, we report a patient who presented with diffuse erythroderma and extensive acantholysis mimicking pemphigus vulgaris histologically.
- Published
- 2019
- Full Text
- View/download PDF
45. Pityriasis rubra pilaris-like erythroderma secondary to phosphoinositide 3-kinase inhibition.
- Author
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Dewan AK, Sowerby L, Jadeja S, Lian C, Wen P, Brown JR, Fisher DC, and LeBoeuf NR
- Subjects
- Aged, Antineoplastic Agents therapeutic use, Dermatitis, Exfoliative pathology, Female, Humans, Leukemia, Lymphocytic, Chronic, B-Cell drug therapy, Male, Middle Aged, Oligodendroglioma drug therapy, Pityriasis Rubra Pilaris pathology, Protein Kinase Inhibitors therapeutic use, Retrospective Studies, Skin pathology, Antineoplastic Agents adverse effects, Dermatitis, Exfoliative chemically induced, Phosphoinositide-3 Kinase Inhibitors, Pityriasis Rubra Pilaris chemically induced, Protein Kinase Inhibitors adverse effects
- Abstract
Background: Phosphoinositide 3-kinase (PI3K) inhibitors are a class of small-molecule inhibitors approved for the treatment of certain leukaemias and lymphomas. Their dermatological adverse event profile is poorly described., Aim: To characterize a rare cutaneous adverse event from PI3K inhibitors in order to help dermatologists and oncologists identify and effectively manage such eruptions., Methods: This was a retrospective analysis of patients receiving PI3K inhibitors referred to the Skin Toxicities Program in The Center for Cutaneous Oncology., Results: Three patients on PI3K inhibitors for treatment of malignancy developed diffuse erythroderma and keratoderma. Clinical and histopathological findings were consistent with pityriasis rubra pilaris (PRP)-like reactions. All patients improved with topical and oral corticosteroids, oral acitretin, and drug discontinuation., Conclusions: PRP-like cutaneous eruptions may develop secondary to PI3K inhibition. Early dermatological evaluation of cutaneous toxicities to PI3K inhibitors as well as rapid initiation of disease-specific treatments may help keep patients on life-prolonging anti-cancer therapies., (© 2018 British Association of Dermatologists.)
- Published
- 2018
- Full Text
- View/download PDF
46. Coinheritance of biallelic SLURP1 and SLC39A4 mutations cause a severe genodermatosis with skin peeling and hair loss all over the body.
- Author
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Harms FL, Nampoothiri S, Kortüm F, Thomas J, Panicker VV, Alawi M, Altmüller J, Yesodharan D, and Kutsche K
- Subjects
- Adolescent, Alopecia diagnosis, Biopsy, Consanguinity, DNA Mutational Analysis, Dermatitis, Exfoliative diagnosis, Dermatitis, Exfoliative pathology, Humans, Keratoderma, Palmoplantar diagnosis, Keratoderma, Palmoplantar pathology, Male, Mutation, Skin pathology, Exome Sequencing, Alopecia genetics, Antigens, Ly genetics, Cation Transport Proteins genetics, Dermatitis, Exfoliative genetics, Keratoderma, Palmoplantar genetics, Urokinase-Type Plasminogen Activator genetics
- Published
- 2018
- Full Text
- View/download PDF
47. Successful treatment of erythrodermic pityriasis rubra pilaris with acitretin.
- Author
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Atzori L, Zanniello R, Lappi A, Pinna AL, Ferreli C, Rongioletti F, and Pilloni L
- Subjects
- Dermatitis, Exfoliative etiology, Dermatitis, Exfoliative pathology, Humans, Male, Middle Aged, Pityriasis Rubra Pilaris complications, Pityriasis Rubra Pilaris pathology, Acitretin therapeutic use, Dermatitis, Exfoliative drug therapy, Keratolytic Agents therapeutic use, Pityriasis Rubra Pilaris drug therapy
- Published
- 2018
- Full Text
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48. Filaggrin 2 Deficiency Results in Abnormal Cell-Cell Adhesion in the Cornified Cell Layers and Causes Peeling Skin Syndrome Type A.
- Author
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Mohamad J, Sarig O, Godsel LM, Peled A, Malchin N, Bochner R, Vodo D, Rabinowitz T, Pavlovsky M, Taiber S, Fried M, Eskin-Schwartz M, Assi S, Shomron N, Uitto J, Koetsier JL, Bergman R, Green KJ, and Sprecher E
- Subjects
- Adult, Aged, Arabs genetics, Biopsy, Cells, Cultured, Codon, Nonsense, Consanguinity, Dermatitis, Exfoliative pathology, Epidermis ultrastructure, Female, Filaggrin Proteins, Homozygote, Humans, Keratinocytes pathology, Male, Microscopy, Electron, Primary Cell Culture, Skin Diseases, Genetic pathology, Exome Sequencing, Cell Adhesion genetics, Dermatitis, Exfoliative genetics, Epidermis pathology, S100 Proteins genetics, Skin Diseases, Genetic genetics
- Abstract
Peeling skin syndromes form a large and heterogeneous group of inherited disorders characterized by superficial detachment of the epidermal cornified cell layers, often associated with inflammatory features. Here we report on a consanguineous family featuring noninflammatory peeling of the skin exacerbated by exposure to heat and mechanical stress. Whole exome sequencing revealed a homozygous nonsense mutation in FLG2, encoding filaggrin 2, which cosegregated with the disease phenotype in the family. The mutation was found to result in decreased FLG2 RNA levels as well as almost total absence of filaggrin 2 in the patient epidermis. Filaggrin 2 was found to be expressed throughout the cornified cell layers and to colocalize with corneodesmosin that plays a crucial role in maintaining cell-cell adhesion in this region of the epidermis. The absence of filaggrin 2 in the patient skin was associated with markedly decreased corneodesmosin expression, which may contribute to the peeling phenotype displayed by the patients. Accordingly, using the dispase dissociation assay, we showed that FLG2 downregulation interferes with keratinocyte cell-cell adhesion. Of particular interest, this effect was aggravated by temperature elevation, consistent with the clinical phenotype. Restoration of corneodesmosin levels by ectopic expression rescued cell-cell adhesion. Taken together, the present data suggest that filaggrin 2 is essential for normal cell-cell adhesion in the cornified cell layers., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)
- Published
- 2018
- Full Text
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49. Erythrodermic pityriasis rubra pilaris managed at home: intensive community care followed by ustekinumab.
- Author
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Ismail N, Callander J, Williams M, and Anstey AV
- Subjects
- Aged, Antibodies, Monoclonal therapeutic use, Community Medicine methods, Dermatitis, Exfoliative pathology, Dermatologic Agents administration & dosage, Dermatologic Agents pharmacology, Dermatologic Agents therapeutic use, Exanthema pathology, Female, House Calls, Humans, Pityriasis Rubra Pilaris pathology, Treatment Outcome, Ustekinumab administration & dosage, Ustekinumab pharmacology, Dermatitis, Exfoliative drug therapy, Exanthema drug therapy, Pityriasis Rubra Pilaris drug therapy, Ustekinumab therapeutic use
- Published
- 2018
- Full Text
- View/download PDF
50. Generalized Ichthyotic Peeling Skin Syndrome due to FLG2 Mutations.
- Author
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Bolling MC, Jan SZ, Pasmooij AMG, Lemmink HH, Franke LH, Yenamandra VK, Sinke RJ, van den Akker PC, and Jonkman MF
- Subjects
- Child, Consanguinity, Dermatitis, Exfoliative pathology, Female, Filaggrin Proteins, Humans, Mutation, Skin pathology, Skin Diseases, Genetic pathology, Dermatitis, Exfoliative genetics, S100 Proteins genetics, Skin Diseases, Genetic genetics
- Published
- 2018
- Full Text
- View/download PDF
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