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Made available in DSpace on 2022-04-29T08:29:31Z (GMT). No. of bitstreams: 0 Previous issue date: 2021-01-01 Departament of Dermatology University of São Paulo Medical School Department of Dermatology University of State of Rio de Janeiro Department of Dermatology Universidade Estadual Paulista Department of Dermatology Clínica Alemana Universidad del Desarollo Department of Dermatology Clínica Universidad de los Andes Department of Dermatology Fundación Valle del Lili Hospital Universitario Universidad ICESI Department of Dermatology and Cutaneous Surgery University of Miami Miller School of Medicine Department of Dermatology Universidade Estadual Paulista

To report our five-years experience on the use of NLRP3 inflammasome functional assays in the differential diagnosis of Brazilian patients with a clinical suspicion of CAPS.The study included 9 patients belonging to 2 families (I, II) and 7 unrelated patients with a clinical suspicion of AID according to Eurofever/PRINTO classification, recruited between 2017 and 2022. The control group for the NLRP3 functional assay consisted of 10 healthy donors and for the CBA cytokines measurement of 19 healthy controls. Patients underwent clinical evaluation, genetic and functional analysis.All members of the family I received the diagnosis of Muckle-Wells Syndrome (MWS), carried the NLRP3 Thr348Met variant and resulted positive for the functional assay. The 2 patients of the family II resulted negative for the mutational screening but positive for the functional assay compatible with a MWS clinical phenotype. In 2 unrelated patients with NLRP3 mutations, including a novel mutation (Gly309Val, Asp303His), a positive functional test confirmed the clinical diagnosis of NOMID. 3 unrelated MWS and 1 FCAS patients resulted negative to the genetic screening and positive for the functional test. One patient with a FCAS-like phenotype harbored the NLRP12 His304Tyr variant confirming the diagnosis of FCAS2.The NLRP3 inflammasome functional assay can assist the clinical diagnosis of CAPS even in patients with unknown genetic defects.

Objective To report our five-years experience on the use of NLRP3 inflammasome functional assays in the differential diagnosis of Brazilian patients with a clinical suspicion of CAPS. Patients and methods: The study included 9 patients belonging to 2 families (I, II) and 7 unrelated patients with a clinical suspicion of AID according to Eurofever/PRINTO classification, recruited between 2017 and 2022. The control group for the NLRP3 functional assay consisted of 10 healthy donors and for the CBA cytokines measurement of 19 healthy controls. Patients underwent clinical evaluation, genetic and functional analysis. Results: All members of the family I received the diagnosis of Muckle-Wells Syndrome (MWS), carried the NLRP3 Thr348Met variant and resulted positive for the functional assay. The 2 patients of the family II resulted negative for the mutational screening but positive for the functional assay compatible with a MWS clinical phenotype. In 2 unrelated patients with NLRP3 mutations, including a novel mutation (​​Gly309Val, Asp303His), a positive functional test confirmed the clinical diagnosis of NOMID. 3 unrelated MWS and 1 FCAS patients resulted negative to the genetic screening and positive for the functional test. One patient with a FCAS-like phenotype harbored the NLRP12 His304Tyr variant confirming the diagnosis of FCAS2. Conclusion: The NLRP3 inflammasome functional assay can assist the clinical diagnosis of CAPS even in patients with unknown genetic defects.

Introduction: Lichen planopilaris (LPP) is a primary lymphocytic cicatricial alopecia with 3 recognized clinical variants. Lately, LPP clinical spectrum has expanded with new and overlapping clinical variants. First considered as a subtype of LPP affecting postmenopausal women, the increasing worldwide incidence of FFA including atypical lesions in young female and male suggests a different pathomechanism for this disease. Although LPP-spectrum disorders may share similar histopathological findings, clinical features and prognosis are different. Case Report: A 26-year-old Caucasian male presented with occipital scarring alopecia and pruritus for the last 6 months. The patient had been treated for an associated androgenetic alopecia and superficial recurrent scalp folliculitis over the vertex scalp for the last 5 years. Trichoscopy of the occipital scalp showed mild diffuse erythema, moderate peripilar scaling, and absence of follicular openings, suggestive of a scarring process. The patient underwent an occipital scalp biopsy that confirmed the diagnosis of a LPP-spectrum disorder. Discussion/Conclusion: Both LPP and FFA mostly affect the anterior-mid scalp of females. However, recent reports on FFA also in premenopausal women and men should make physicians aware of atypical features of this disease and unusual clinical presentation.

Hidradenitis suppurativa is a chronic immune mediated disease of universal distribution that causes great damage to the quality of life of the affected individual, whose prevalence is estimated at 0.41% in the Brazilian population. The objective of this work was update on physiopathogenesis, diagnosis and classification of hidradenitis suppurativa and to establish therapeutic recommendations in the Brazilian reality. It was organized as a work group composed of eight dermatologists from several institutions of the country with experience in the treatment of hidradenitis suppurativa and carried out review on the topic. Recommendations were elaborated and voted by modified Delphi system and statistical analysis of the results was performed. The Brazilian consensus on the clinical approach of hidradenitis suppurativa had the support of the Brazilian Society of Dermatology.

Lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) are chronic lymphocytic cicatricial alopecias (CAs) with unclear etiopathogenesis. While LPP usually presents as inflamed and quickly progressing lesions over the vertex, FFA tends to affect primarily women as a slowly progressive frontotemporal alopecia. Both diseases can be associated with certain nonscalp lesions that may even precede the scalp lesions,(1-4) and be very distressing, leading to impaired self-esteem and quality of life (QoL).(5-7) We aimed to assess the relationship between QoL, signs and symptoms of inflammation, and associated nonscalp lesions in LPP and FFA.

Objective The aim of this study was to identify the pattern of pediatric dermatoses of patients evaluated at a dermatologic clinic of a reference center in Brazil and to compare these results to similar surveys conducted in other countries. Methods A retrospective study was performed of patients up to 18 years old, evaluated at a dermatologic clinic between January 1, 2017 and December 31, 2017. Variables collected for analysis included age, gender, dermatological diagnosis, multidisciplinary follow-up, hospitalization, and complementary exams. Results A total of 2330 patients were included for analysis, with a mean age of 9.7 years. 295 patients were diagnosed with more than one skin disease, leading to a total of 2668 diagnoses. Skin diseases were organized into categories and inflammatory dermatoses corresponded to the largest group (31.2%), mostly due to atopic dermatitis (18.3%). The other main categories were: genodermatoses (14.2%), infectious diseases (12.6%), adnexal disorders (12.5%), cysts and neoplasms (10.7%), and vascular disorders (7.0%). Fifty-six patients needed to be admitted to the dermatology ward; 25 of them (44.6%) for management of worsening of the skin disease, mainly atopic dermatitis, psoriasis, and drug reactions. There were 885 biopsies performed in 38.0% of the subjects and 751 patients (32.2%) required multidisciplinary care; most of them had some genodermatoses. Conclusions Dermatologic disorders are very common in the pediatric age group and differ from those in adults, suffering influence from cultural, ethnic, socioeconomic, and environmental factors. Knowing the magnitude and distribution of these dermatoses is important to better plan healthcare policies.

Sideroblastic anaemia, B-cell immunodeficiency, periodic fever and developmental delay (SIFD) is caused by mutations of TRNT1, an enzyme essential for mitochondrial protein synthesis, and has been reported in 23 cases. A 6-month-old girl was evaluated with recurrent fever, failure to thrive, skin lesions and anaemia. She received blood transfusions and empirical antibiotics. Skin lesions, previously interpreted as insect bites, consisted of numerous firm asymptomatic erythematous papules and nodules, distributed over trunk and limbs. Skin histopathology revealed an intense dermal neutrophilic infiltrate extending to the subcutaneous, with numerous atypical myeloid cells, requiring the diagnosis of leukaemia cutis, to be ruled out. Over the follow-up, she developed herpetic stomatitis, tonsillitis, lobar pneumonia and Metapneumovirus tracheitis, and also deeper skin lesions, resembling panniculitis. Hypogammaglobulinaemia was diagnosed. An autoinflammatory disease was confirmed by whole exome sequencing: heterozygous mutations for TRNT1 NM_182916 c.495_498del, p.F167Tfs * 9 and TRNT1 NM_182916 c.1246A>G, p.K416E. The patient has been treated with subcutaneous immunoglobulin and etanercept. She presented with developmental delay and short stature for age. The fever, anaemia, skin neutrophilic infiltration and the inflammatory parameters improved. We describe a novel mutation in SIFD and the first to present skin manifestations, namely neutrophilic dermal and hypodermal infiltration.

Folliculitis decalvans (FD) is a neutrophilic primary scarring alopecia characterized by chronic inflammation and intense fibrosis. It usually affects the vertex and occipital scalp of young males with recurrent outbreaks and poor therapeutic outcomes.1 In this study, our goal was to describe clinical, histopathological and immunohistochemical features of the novel scalp blue lesions in patients with FD. A descriptive cross-sectional study included seven patients with FD biopsy-proven seen at the University of Sao Paulo from 2018 to 2020.

Background: Acne in women is often associated with anxiety and depression, and may persist from adolescence as well as manifest for the first time in adulthood. Genetic and hormonal factors contribute to its etiopathogenesis, and maintenance treatment is required, usually for years, due to its clinical evolution. Objective: To develop a guide for the clinical practice of adult female acne. Methods: A team of five experts with extensive experience in acne conducted a literature review of the main scientific evidence and met to discuss the best practices and personal experiences to develop a guide containing recommendations for the clinical practice of adult female acne. Results: The group of specialists reached consensus on the main guidelines for clinical practice, providing detailed recommendations on clinical picture, etiopathogenesis, laboratory investigation and treatment of adult female acne. Conclusion: Different from teenage acne, adult female acne presents some characteristics and multiple etiopathogenic factors that make its management more complex. This guide provides recommendations for best clinical practices and therapeutic decisions. However, the authors consider that additional studies are needed in order to provide more evidence for adult female acne to be better understood.

BACKGROUND: Atopic dermatitis is a highly prevalent inflammatory and pruritic dermatosis with a multifactorial etiology, which includes skin barrier defects, immune dysfunction, and microbiome alterations. Atopic dermatitis is mediated by genetic, environmental, and psychological factors and requires therapeutic management that covers all the aspects of its complex pathogenesis. OBJECTIVES: The aim of this article is to present the experience, opinions, and recommendations of Brazilian dermatology experts regarding the therapeutic management of atopic dermatitis. METHODS: Eighteen experts from 10 university hospitals with experience in atopic dermatitis were appointed by the Brazilian Society of Dermatology to organize a consensus on the therapeutic management of atopic dermatitis. The 18 experts answered an online questionnaire with 14 questions related to the treatment of atopic dermatitis. Afterwards, they analyzed the recent international guidelines on atopic dermatitis of the American Academy of Dermatology, published in 2014, and of the European Academy of Dermatology and Venereology, published in 2018. Consensus was defined as approval by at least 70% of the panel. RESULTS/CONCLUSION: The experts stated that the therapeutic management of atopic dermatitis is based on skin hydration, topical anti-inflammatory agents, avoidance of triggering factors, and educational programs. Systemic therapy, based on immunosuppressive agents, is only indicated for severe refractory disease and after failure of topical therapy. Early detection and treatment of secondary bacterial and viral infections is mandatory, and hospitalization may be needed to control atopic dermatitis flares. Novel target-oriented drugs such as immunobiologicals are invaluable therapeutic agents for atopic dermatitis.

Made available in DSpace on 2019-10-04T12:38:20Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-03-01. Added 1 bitstream(s) on 2019-10-09T18:34:12Z : No. of bitstreams: 1 S0365-05962019000700056.pdf: 486399 bytes, checksum: a2e0a3f4581e4f634c104b420135972a (MD5) Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) BACKGROUND: Urticarias are frequent diseases, with 15% to 20% of the population presenting at least one acute episode in their lifetime. Urticaria are classified in acute ( 6 weeks). They may be induced or spontaneous. OBJECTIVES: To verify the diagnostic and therapeutic recommendations in chronic spontaneous urticaria (CSU), according to the experience of Brazilian experts, regarding the available guidelines (international and US). Methods: A questionnaire was sent to Brazilian experts, with questions concerning diagnostic and therapeutic recommendations for CSU in adults. RESULTS: Sixteen Brazilian experts answered the questionnaire related to diagnosis and therapy of CSU in adults and data were analyzed. Final text was written, considering the available guidelines (International and US), adapted to the medical practices in Brazil. Diagnostic work up in CSU is rarely necessary. Biopsy of skin lesion and histopathology may be indicated to rule out other diseases, such as, urticarial vasculitis. Other laboratory tests, such as complete blood count, CRP, ESR and thyroid screening. Treatment of CSU includes second-generation anti-histamines (sgAH) at licensed doses, sgAH two, three to fourfold doses (non-licensed) and omalizumab. Other drugs, such as, cyclosporine, immunomodulatory drugs and immunosuppressants may be indicated (non-licensed and with limited scientific evidence). CONCLUSIONS: Most of the Brazilian experts in this study partially agreed with the diagnostic and therapeutic recommendations of the International and US guidelines. They agreed with the use of sgAH at licensed doses. Increase in the dose to fourfold of sgAH may be suggested with restrictions, due to its non-licensed dose. Sedating anti-histamines, as suggested by the US guideline, are indicated by some of the Brazilian experts, due to its availability. Adaptations are mandatory in the treatment of CSU, due to scarce or lack of other therapeutic resources in the public health system in Brazil, such as omalizumab or cyclosporine. Univ Sao Paulo, Dermatol, Fac Med, Sao Paulo, SP, Brazil Fac Med ABC, Postgrad Program, Sao Paulo, SP, Brazil Univ Sao Paulo, Dept Dermatol, Fac Med, Sao Paulo, SP, Brazil Univ Fed Pernambuco, Recife, PE, Brazil Univ Nove Julho, Dermatol, Sao Paulo, SP, Brazil Univ Fed Minas Gerais, Dermatol Serv, Hosp Clin, Belo Horizonte, MG, Brazil Univ Fed Minas Gerais, Dermatol, Fac Med, Belo Horizonte, MG, Brazil Univ Fed Pernambuco, Dept Dermatol, Ctr Ciencias Med, Fac Med, Recife, PE, Brazil Fac Evangel Curitiba, Dermatol Serv, Curitiba, Parana, Brazil Univ Sao Paulo, Div Dermatol, Hosp Clin, Fac Meclicina, Sao Paulo, SP, Brazil Univ Estadual Paulista, Dept Dermatol & Radiotherapy, Botucatu, SP, Brazil Hosp Padre Bento Guarulhos, Training & Improvement Management, Sao Paulo, SP, Brazil Hosp Serv Publ Estadual Sao Paulo, Dermatol Serv, Sao Paulo, SP, Brazil Univ Fed Sao Paulo, Urticaria Outpatient Clin, Dept Dermatol, Sao Paulo, SP, Brazil Univ Fed Rio Grande do Sul, Dermatol Serv, Hosp Clin Porto Alegre, Fac Med, Porto Alegre, RS, Brazil Ambulatorio Dermatol Sanitaria, Porto Alegre, RS, Brazil Santa Casa Porto Alegre, Dermatol Serv, Porto Alegre, RS, Brazil Santa Casa Misericordia Sao Paulo, Dermatol Clin, Sao Paulo, SP, Brazil Santa Casa Misericardia Sao Paulo, Dermatol, Fac Ciencias Med, Sao Paulo, SP, Brazil Fac Med ABC, Sao Paulo, SP, Brazil Univ Estadual Paulista, Dept Dermatol & Radiotherapy, Botucatu, SP, Brazil CAPES: 001

Background Infantile hemangiomas (IHs) are the most common benign vascular tumors of childhood. Propranolol is an effective drug in treating IH. A reliable and complementary instrument is necessary to evaluate IH response to propranolol in addition to clinical and photographic assessments. Ultrasonography is a simple and non-invasive technique that enables precise measurements of tumor size and contributes to objective follow-up. Objective To demonstrate the use of serial ultrasonography as an adjunctive tool for assessment of IH treatment with propranolol. Patients and methods A retrospective study of 19 patients with IH treated with propranolol was conducted from January 2009 to March 2014. Data of individual IH volume at the beginning and at least 6 months after the onset of treatment and overall volume reduction by ultrasonographic measurement were obtained. Results We observed a statistically significant IH volume reduction of approximately 0.51 cm3. This volume corresponds to an average reduction of 47% in the final volume compared with the initial volume. Conclusion Ultrasonographic measurements contribute to demonstrate tumor regression and IH response to propranolol. Thus, ultrasonography is an important instrument to guide therapeutic strategies.

Introduction Harlequin ichthyosis (HI) is a severe and rare hereditary congenital skin disorder characterized by excessive dryness, ectropion and eclabion. The association of ichthyosis with systemic sclerosis has been described in only three children. No patient with generalized morphea (GM) associated with harlequin ichthyosis was described. Case report A 4-years and 6-months girl, diagnosed with harlequin ichthyosis based on diffuse cutaneous thickening, scaling, erythema, ectropion and eclabium since the first hours of birth was described. She was treated with acitretin (1.0 mg/kg/day) and emollient cream. At 3 years and 9 months, she developed muscle contractures with pain on motion and limitation in elbows and knees, and diffuse sclerodermic plaques on the abdomen, back, suprapubic area and lower limbs. Skin biopsy showed rectified epidermis and mild hyperorthokeratosis, reticular dermis with perivascular and periadnexal infiltrates of lymphocytes and mononuclear cells, and reticular dermis and sweat gland sclerosis surrounded by a dense collagen tissue, compatible with scleroderma. The patient fulfilled the GM subtype criteria. Methotrexate and prednisone were introduced. At 4 years and 3 months, new scleroderma lesions occurred and azathioprine was associated with previous therapy, with no apparent changes after two months. Discussion A case of harlequin ichthyosis associated with a GM was reported. The treatment of these two conditions is a challenge and requires a multidisciplinary team.

ResumoIntroduçãoIctiose arlequim é uma doença cutânea congênita grave, autossômica e rara, caracterizada por ressecamento excessivo da pele e hiperqueratose. A associação de ictiose com esclerose sistêmica foi descrita em apenas três crianças. Ainda não foi descrito nenhum paciente com morfeia generalizada (MG) associada à ictiose arlequim.Relato de casoMenina de quatro anos e seis meses de idade com diagnóstico de ictiose arlequim baseado em espessamento cutâneo difuso, com fissuras, descamação, eritema e sangramento da lesão desde as primeiras horas de vida. A paciente foi tratada com acitretina (1,0mg/kg/dia) e creme emoliente. Aos três anos e nove meses, desenvolveu contraturas musculares com dor à movimentação e limitação nos cotovelos e joelhos e placas esclerodérmicas difusas no abdômen, nas costas, na região suprapúbica e nas extremidades inferiores. A biópsia de pele mostrou epiderme retificada e hiperqueratose leve, derme reticular com linfócitos, infiltrado mononuclear perivascular e perianexial e esclerose da derme reticular e glândula sudorípara rodeada por um tecido colágeno denso, compatível com esclerodermia. A paciente preencheu os critérios para o subtipo MG. Metotrexato e prednisona foram introduzidos. Aos quatro anos e três meses, apresentou novas lesões esclerodérmicas, associando‐se azatioprina à terapêutica anterior, sem resposta após dois meses.DiscussãoUm caso de ictiose arlequim associada à MG foi descrito. O tratamento dessas duas condições é um desafio e requer uma equipe multidisciplinar.AbstractIntroductionHarlequin ichthyosis (HI) is a severe and rare hereditary congenital skin disorder characterized by excessive dryness, ectropion and eclabion. The association of ichthyosis with systemic sclerosis has been described in only three children. No patient with generalized morphea (GM) associated with harlequin ichthyosis was described.Case reportA 4‐year and 6‐months girl, diagnosed with harlequin ichthyosis based on diffuse cutaneous thickening, scaling, erythema, ectropion and eclabium since the first hours of birth was described. She was treated with acitretin (1.0mg/kg/day) and emollient cream. At 3 years and 9 months, she developed muscle contractures with pain on motion and limitation in elbows and knees, and diffuse sclerodermic plaques on the abdomen, back, suprapubic area and lower limbs. Skin biopsy showed rectified epidermis and mild hyperorthokeratosis, reticular dermis with perivascular and periadnexal infiltrates of lymphocytes and mononuclear cells, and reticular dermis and sweat gland sclerosis surrounded by a dense collagen tissue, compatible with scleroderma. The patient fulfilled the GM subtype criteria. Methotrexate and prednisone were introduced. At 4 years and 3 months, new scleroderma lesions occurred and azathioprine was associated with previous therapy, with no apparent changes after two months.DiscussionA case of harlequin ichthyosis associated with a GM was reported. The treatment of these two conditions is a challenge and requires a multidisciplinary team.

Background The Quality of Life Evaluation in Epidermolysis Bullosa (QoLEB) questionnaire was developed for use in English-speaking individuals. Objectives The aims of this study were to translate the QoLEB into Brazilian Portuguese, to culturally adapt it, and to verify its reliability and validity. Methods The study followed the steps proposed by the World Health Organization, which include: translation; evaluation by a panel of experts and patients; back translation; and linguistic and cultural adaptation. All subjects were examined and assessed using the QoLEB and the Dermatology Life Quality Index (DLQI) or the Children's Dermatology Life Quality Index (CDLQI). Translation and cultural and linguistic adaptation were conducted by 10 patients, three translators, and the bilingual authors. Results The population was composed of 40 children (mean age: 8,15 years) and 17 adults (mean age: 33 years). Among the children, eight had epidermolysis bullosa (EB) simplex (EBS), one had junctional EB (JEB), and 31 had dystrophic EB (DEB). In the adult group, four of the subjects had EBS, one had JEB, and 12 had DEB. Mean ± standard deviation (SD) scores on the QoLEB in children were 10.60 ± 7.13 in EBS subjects, 9.71 ± 7.87 in children with dominant DEB (DDEB), and 14.25 ± 9.67 in children with recessive DEB (RDEB). Mean ± SD scores in adults were 12.50 ± 9.95 in EBS subjects, 12.00 ± 5.83 in DDEB subjects, and 20.20 ± 9.21 in RDEB subjects. The QoLEB-BP (Brazilian Portuguese) showed high internal consistency (Cronbach's α = 0.88) and high test–retest reliability (intraclass correlation coefficient: 0.70), confirming the internal consistency and reproducibility of this Portuguese version. There were significant correlations between QoLEB scores and both CDLQI (Pearson's r = 0.688, P < 0.002) and DLQI (Pearson's r = 0.807, P < 0.001) scores. Conclusions Epidermolysis bullosa has marked impacts on the lives of EB patients and their families, which are strongly correlated with disease severity. The Brazilian Portuguese version of the QoLEB is validated and can be recommended for use in subsequent studies.

Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.

Rosacea is a chronic inflammatory condition with predominant facial involvement. Because of that, many patients sense that rosacea affects quality of life. The etiology of rosacea remains unknown. Recent studies have suggested that aberrant innate immunity is central to this disease. The aim of this study was to examine the presence of Langerhans cells, plasmacytoid dentritic cells (PDC), the expression of Toll-like receptors (TLR) and inducible oxide nitric synthase (iNOS) in skin of patients with rosacea, to highlight the participation of innate immunity in its pathogenesis. 28 biopsy specimens were taken from patients with clinical and histopathological findings of rosacea. Immunohistochemical demonstration of Langerhans cells (anti-CD1a antibody), PDC (anti-CD 123 antibody), TLR2, TLR4 and iNOS was performed in skin samples and compared with normal skin controls. The expression of Langerhans cells was lower in rosacea group than in control group. PDC were found in skin samples of rosacea as isolated cells and forming small clusters. Expression of TLR2, TLR4 and iNOS was higher in rosacea samples than in normal skin controls. This research demonstrates early and late stage components of innate immunity in specimens of rosacea ratifying the existence of an altered innate immunity in its pathogenesis.

Nevus lipomatosus cutaneous superficialis (NLCS) is a rare cutaneous hamartoma characterized by groups of mature fat cells in the dermis. The authors report a case of a dark-skinnned, 6-year-old, Brazilian girl with 1 lesion in the right upper chest, extending to the neck, since 1 year of age. The dermatological examination consisted of skin-colored and yellowish follicular papules, isolated and grouped into a papillomatous plaque. The biopsy revealed ectopic mature adipose tissue in the dermis, compatible with NLCS, perifollicular well-circumscribed fibroblast and collagen proliferation, and decreased elastic fibers in the papillary and upper reticular dermis. The finding of perifollicular fibrosis in NLCS is not common, having been first described in 2003. The perifollicular fibrosis is characteristic of other rare hamartoma, the perifollicular fibroma, but is also present in fibrofolliculomas or trichodiscomas, angiofibromas, and fibrotic papules on the face of tuberous sclerosis and postinflammatory fibrosis. This case is very similar to the first described case of NLCS with perifollicular fibrosis, and the authors believe that this is the second case of this entity, but they propose to name it NCLS with perifollicular fibromas, which better describes the nature of this lesion.

Summary Background Epidermolysis bullosa (EB) nevi are acquired pigmented melanocytic lesions which may have clinical and dermoscopic features quite similar to those found in melanoma. More detailed information on this phenomenon is still lacking. Objectives To evaluate clinical, dermoscopic, and histopathological features of melanocytic lesions in 13 patients with dystrophic EB (DEB). Patients and Methods Patients underwent clinical and dermoscopic evaluation. Suspicious lesions were excised and examined microscopically. Results There were 12 cases of recessive DEB and one of dominant DEB. Five patients were men; 8 were women; the ages ranged from 2 to 27 years old. All patients had at least 2 atypical melanocytic lesions. Two of the 5 biopsied patients showed an atypical nevus or lentigo on histopathological examination. Conclusions We observed a high incidence of large and atypical melanocytic lesions in DEB patients. Although the exact explanation for this is still unclear, it seems that re-epithelization and the chronic inflammatory process may stimulate the proliferation of melanocytes, as well as the emergence of lesions with atypical clinical and dermoscopic features. As an unequivocal discrimination from malignant melanoma in vivo is not always possible, regular clinical follow-up and histopathological evaluation of suspicious lesions in EB patients are mandatory.

As lesões melanocíticas adquiridas podem apresentar aspecto clínico não-usual em pacientes portadores de epidermólise bolhosa hereditária. Essas lesões são conhecidas como "nevos EB" e, muitas vezes, constituem um desafio diagnóstico ao dermatologista por apresentarem características clínicas, dermatoscópicas e histopatológicas semelhantes às encontradas no melanoma. Não são exclusivas de nenhuma forma de epidermólise bolhosa e têm sua frequência aumentada na infância. Relata-se o caso de um doente do sexo masculino, de 6 meses de idade, portador da forma distrófica recessiva da doença, com lesão pigmentada de rápido crescimento na coxa esquerda. Optou-se por seguimento clínico da lesão, considerando que os aspectos clínicos, dermatoscópicos e histológicos eram compatíveis com a descrição de outros casos de nevo EB previamente descritosAcquired melanocytic lesions may present unusual clinical features in all forms of hereditary epidermolysis bullosa. These lesions are known as "EB nevi", and often pose a diagnostic challenge for dermatologists given their resemblance - clinically, dermoscopically and histologically - to melanoma. The lesions have been reported in all types of hereditary EB, most of them in childhood. We report the case of a 6-month-old boy suffering from recessive dystrophic epidermolysis bullosa (RDEB) that presented as a large pigmented lesion on his left thigh. We decided to monitor the lesion closely since we considered that the clinical and pathological aspects of the lesion were compatible with the description of other previously reported cases of EB nevi

A pele do neonato é submetida a um progressivo processo de adaptação ao ambiente extrauterino, para o qual cuidados especiais se tornam necessários. A sua pele caracteriza-se por ser sensível, fina e frágil. A imaturidade da sua barreira epidérmica diminui significativamente a defesa contra a excessiva proliferação microbiana, torna a pele mais susceptível ao trauma e à toxicidade por absorção percutânea de drogas. Devido às características próprias da pele do recém-nascido (RN), de lactentes e de crianças, o uso dos produtos cosméticos destinados à sua higiene e proteção requer um cuidado especial. Com o intuito de preservar a integridade da pele neonatal e infantil, este artigo revisou os cuidados preventivos básicos que se devem ter com a pele dos bebês quanto à higiene, ao banho, ao uso de agentes de limpeza, a produtos tópicos e a sua toxicidade percutânea.Neonatal skin suffers a progressive adaptation to the extrauterine environment and special care is needed during this period. This skin is very sensitive, thin and fragile. Immaturity of the epidermal barrier reduces the defense against the excessive proliferation of microbes and makes the skin more vulnerable to trauma and percutaneous drug toxicity. Because of the peculiar characteristics of newborn, infant and children's skin, the use of cosmetic products designed for hygiene and protection requires caution. In order to preserve the integrity of neonatal and child's skin, this article reviewed basic preventive care practices in relation to hygiene, bathing, cleansing agents, topical products and their percutaneous toxicity.

A pele do neonato é submetida a um progressivo processo de adaptação ao ambiente extrauterino, para o qual cuidados especiais se tornam necessários. A sua pele caracteriza-se por ser sensível, fina e frágil. A imaturidade da sua barreira epidérmica diminui significativamente a defesa contra a excessiva proliferação microbiana, torna a pele mais susceptível ao trauma e à toxicidade por absorção percutânea de drogas. Devido às características próprias da pele do recém-nascido (RN), de lactentes e de crianças, o uso dos produtos cosméticos destinados à sua higiene e proteção requer um cuidado especial. Com o intuito de preservar a integridade da pele neonatal e infantil, este artigo revisou os cuidados preventivos básicos que se devem ter com a pele dos bebês quanto à higiene, ao banho, ao uso de agentes de limpeza, a produtos tópicos e a sua toxicidade percutânea.

Ichthyosis is a heterogeneous cornification disorder. Melanocytic lesions have not been previously described in association with ichthyosis. Their clinical importance lies in the fact that they may simulate melanoma clinically and dermoscopically, as seen in epidermolysis bullosa. The objective of this study was to evaluate the clinical, dermoscopic, and histopathologic features of nevi and lentigines in 16 patients with autosomal recessive congenital ichthyosis-lamellar ichthyosis and nonbullous ichthyosiform congenital erythroderma. Patients underwent general clinical examination dermoscopy. The more suspicious lesions were excised and to histopathologic examination. Most patients (n = 13) reported no personal or familial history of melanoma or atypical nevi. All of the patients had at least five atypical melanocytic lesions. Ten of the 16 patients had at least one atypical nevus or lentigo. This study suggests that increased atypical melanocytic nevi may be a feature of long-standing congenital ichthyoses. Whether this finding is disease-related or a coincidental observation is difficult to ascertain. As an unequivocal discrimination from malignant melanoma in vivo is not always possible, regular clinical follow-up of patients with ichthyosis and increased or unusual nevi is recommended.

A dermatite da área da fralda irritativa primária é a mais prevalente desse tipo, sendo provavelmente a afecção cutânea mais freqüente na primeira infância. Constitui fonte significativa de desconforto para a criança. O uso da fralda ocasiona aumento da temperatura e da umidade locais. Há conseqüente maceração da pele, que se torna mais susceptível à irritação ocasionada pelo contato prolongado da urina e das fezes. Freqüentemente surge infecção secundária por Candida albicans ou por bactérias como Bacillos faecallis, Proteus, Pseudomonas, Staphylococcus e Streptococcus. O uso de pós, óleos, sabões e pomadas irritantes agravam o quadro clínico. A melhor conduta é a prevenção que engloba um conjunto de medidas que têm como objetivos manter a superficie seca, limitar a mistura e dispersão da urina e das fezes, reduzir seu contato com a pele, evitar irritação e maceração, preservar a função de barreira cutânea e manter, sempre que possível, um pH ácido. O tratamento médico consiste em medidas simples, ajustadas de acordo com a gravidade e o tipo de dermatite.