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Pruritus is the main symptom of many dermatologic and systemic diseases. Atopic dermatitis, psoriasis, contact dermatitis, urticaria, lichen simplex chronicus, mycosis fungoides, scars, autoimmune diseases, kidney or liver diseases among others are all associated with itch that may require different approaches to management. Although antihistamines seem to be the first line of therapy, in reality their role is limited to urticaria and drug-induced reactions. In fact, the pathophysiologic mechanisms of each of the conditions covered in this review will differ. Recent years have seen the emergence of new drugs whose efficacy and safety profiles are very attractive for the management of pruritus in clinical practice. Clearly we are at a critical moment in dermatology, in which we have the chance to be more ambitious in our goals when treating patients with pruritus., (Copyright © 2023 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)

Significant racial/ethnic disparities in dermatologic care and their subsequent impact on dermatologic conditions were recently reported. Contributing factors include socioeconomic factors, gaps in educational exposure, and underrepresentation of minority groups in the dermatologic workforce. In 2021, the American Academy of Dermatology (AAD) announced its three-year plan to expand diversity, equity, and inclusion in dermatology. One way to reduce disparities in dermatology is for every dermatologist, regardless of race or ethnicity, to receive adequate education in diseases, treatments, health equity, and tailored approaches to delivering dermatologic care with cultural humility. In addition, a diverse dermatologic workforce-especially at the level of residency program educators and organizational leaders-will contribute to improved cross-cultural understanding, more inclusive research efforts, and improved treatment approaches for conditions that are more prevalent or nuanced in certain racial/ethnic populations. Finally, the dermatology and broader healthcare community needs to acknowledge and educate ourselves on the health impacts of racism., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background and Objective: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years., Patients and Methods: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years., Results: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%])., Conclusion: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data., (Copyright © 2022 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background/objectives: Primary cutaneous lymphomas are rare in pediatric patients. The clinical and histopathological manifestations may differ from those in adults. Due to their low frequency and the insidious clinical picture, the diagnosis is usually delayed. The Spanish Primary Cutaneous Lymphoma Registry was initiated in 2016 as a multicenter registry that would allow better insight into the epidemiological, clinical, histopathological, and treatment response characteristics of patients with primary cutaneous lymphomas., Methods: We conducted a prospective observational cohort study of primary cutaneous lymphomas in pediatric patients participating in the Spanish Academy of Dermatology and Venereology (AEDV) Primary Cutaneous Lymphoma Registry., Results: At the time of the analysis, 10 patients under 18 years of age out of 799 all-age cases (1.25%) had been included in the registry (7 males, 3 females). The mean age at diagnosis was 9.7 years (SD: 4.8). Seven (70%) had mycosis fungoides, 2 of them had the folliculotropic variant; and 3 (30%) had primary cutaneous marginal zone B-cell lymphoma., Conclusions: Primary cutaneous lymphomas are extremely rare in pediatric patients and usually have a good prognosis. Therefore, a high level of suspicion is necessary for the diagnosis. We suggest management by experienced physicians and follow-up into adulthood., (© 2021 Wiley Periodicals LLC.)

Palliative care has been shown to improve quality of life, symptoms, and caregiver burden for a range of life-limiting diseases. Palliative care use among patients with severe dermatologic disease remains relatively unexplored, but the limited available data suggest significant unmet care needs and low rates of palliative care use. This review summarizes current palliative care patterns in dermatology, identifying areas for improvement and future investigation., (Copyright © 2020. Published by Elsevier Inc.)

Background: Given the severe symptom burden and chronic nature of mycosis fungoides (MF) and Sézary syndrome (SS), effective assessment of quality of life (QoL) is essential to guiding patient-centred care in this population. In this study, we aim to provide a comprehensive assessment of QoL in early- and advanced-stage MF/SS and to assess the correlation of traditional measures of clinical severity with QoL measures., Methods: Between July 2017 and April 2019, outpatients at an academic medical centre with either MF/SS (n = 115) or general dermatology concerns (n = 115) completed generic and dermatology-specific QoL instruments [Health Utilities Index Mark 3 (HUI3), RAND 36-Item Short-Form Health Survey (SF-36), Skindex-29, visual analogue scale for itch (VAS itch) and 5-D pruritus scale]. The mean scores of MF/SS patients were compared to that of controls using multivariable regression models adjusted for demographics and medical comorbidities. Cluster analysis of the QoL instruments and clinical severity measures (e.g. stage and body-surface-area involvement) was performed., Results: Patients with MF/SS scored significantly worse than controls on all QoL instruments used, with advanced-stage (IIB-IVB) disease having the worst QoL impairment. Early-stage (IA-IIA) and advanced-stage MF/SS patients had significantly reduced overall health status (HUI3; P < 0.05), with largest decrements in social functioning and usual role functioning due to physical and emotional health (SF-36; all P < 0.05). MF/SS had significantly worse skin-specific impairment than controls, with advanced-stage disease reporting the most severe skin-specific burden (Skindex-29, P < 0.05). Clinical severity measures had a weak correlation with generic (|r s | = 0.02-0.27) and moderate correlation with dermatology-specific instruments (|r s | = 0.41-0.53)., Conclusions: MF/SS have a significant impact on multiple domains of patients' QoL, including social, emotional and physical functioning. Current clinical measures do not adequately address QoL outcomes, underscoring the need for integrating formal disease-specific QoL assessment into the routine evaluation of MF/SS patients., (© 2019 European Academy of Dermatology and Venereology.)

Primary cutaneous lymphomas represent a broad group of diseases with different clinical, histopathological, phenotypic, molecular, and prognostic features. All cutaneous lymphomas share the same tropism of neoplastic lymphocytes for the skin, but precise classification is paramount for proper management of the patients. Primary cutaneous lymphomas are classified according to the schemes proposed by the European Organization for Research and Treatment of Cancer (EORTC)-Cutaneous Lymphomas Task Force together with the World Health Organization (WHO) in 2005, and the WHO classification of 2008 with the 2016 update. The recognition of organ-based lymphomas, including cutaneous lymphomas, reflects a shift in the approach to lymphoproliferative disorders, and represents one of the major advances in the WHO classification of hematological tumors. Future studies should be aimed at shedding light on the many grey areas of cutaneous lymphomas (particularly the diagnosis and nomenclature of early mycosis fungoides and variants), and at gathering more data on the disorders that are still listed as provisional entities in the WHO classification., (Copyright © 2017 Elsevier Inc. All rights reserved.)

The use of ionizing radiation and corticosteroids is discussed, in this third and final part of a review of diseases of the skin. Radiation is being used less extensively because superior methods of treatment are available for many conditions which formerly were frequently treated by this modality. The concept of applying the radiation at the level of the basic pathologic process has been developed into clinical practice by the use of generators which can produce very soft (or superficial) ionizing radiation. Topical or systemic corticosteroids do not cure skin diseases but produce dramatic suppression of signs and symptoms. For best results consideration must be given to the diagnosis, the natural history of the disease to be treated, the method of administration and a search for possible contraindications to the use of these steroids. Basic dermatological principles (removal of offending agents, bland soothing applications, sedation, etc.) must be adhered to. The corticosteroids are not a panacea in the treatment of skin disease.

Abstract Introduction Diagnosis of persistent erythematous, scaly patches, or plaques can be complex since psoriasis (Ps), eczematous dermatitis (ED), and mycosis fungoides (MF) can be considered. Dermoscopy, which is a noninvasive diagnostic tool, is commonly used to examine blood vessels, scales, and background color; however, research on hair shaft evaluation in inflammatory dermatoses remains scarce. The aim of the study was dermoscopic evaluation of hair shafts in skin lesions localized on the non-scalp skin areas in patients diagnosed with MF, Ps, and ED. Methods This was a retrospective evaluation of 55 patients diagnosed with MF, Ps, and ED. Photographic and dermoscopic documentation of these patients and detailed medical history were evaluated. Results A total of 21 patients with MF, 21 patients with Ps, and 13 patients with ED were evaluated. The examination revealed the presence of various abnormalities of hair shafts (e.g., numerous pili torti, single pili torti, 8-shaped hairs, pigtail hairs, broken hairs, hair shafts rapidly tapered over long sections, hair shafts irregular in thickness, angulated hairs, branched hairs, the presence of trichorrhexis nodosa, and monilethrix-like hairs), yellow dots, and black dots. The presence of pili torti was found in 80% of patients with MF, compared with 16% of patients with Ps and 8% of patients with ED (p

Primary cutaneous lymphomas (PCLs), especially mycosis fungoides (MF), pose significant diagnostic and therapeutic challenges. This study aims to correlate initial histological features with the disease course and survival in MF patients. A retrospective–prospective cohort study was conducted on 83 patients diagnosed with early-stage MF at the Departments of Dermatovenerology and Pathology, UHC Zagreb, from January 2003 to December 2012. The analyzed histopathological parameters included lichenoid dermal lymphocyte infiltrate, Pautrier microabscesses, and lymphocyte atypia. Patients with more than 30 guardian lymphocytes per 100 keratinocytes exhibited worse overall and progression-free survival. Furthermore, those with over 50% atypical lymphocytes demonstrated a faster progression rate. A dense lichenoid dermal infiltrate and a high count of lymphocyte “keepers” significantly increased the mortality risk within five years of diagnosis. This study did not fully confirm the hypothesis regarding the prognostic value of large Pautrier microabscesses but highlighted the importance of dense lichenoid infiltrates. The study identified new potential histopathological prognostic factors in early-stage MF, suggesting the need for larger studies to confirm these findings. The identification of such predictors could enhance the prognostic stratification and guide more tailored therapeutic approaches for MF patients.

Abstract Cutaneous T‐cell lymphomas (CTCLs) are unique non‐Hodgkin lymphomas due to the proliferation of skin‐homing T cells. Sezary syndrome (SS), a subtype of CTCL, typically presents as erythroderma with specific blood involvement and seldomly evolves from tumor‐stage mycosis fungoides (MF). The article examines the case of a 62‐year‐old female patient who developed SS while under atezolizumab treatment for tumor‐stage transformed MF. Postdiagnosis, she underwent treatments including topical corticosteroids, phototherapy, and other drug regimens, experiencing both complete responses and relapses over the years. Three years postdiagnosis, her condition deteriorated with facial tumor lesions, confirmed as MF. Despite multiple treatments, including oral drugs, radiation therapy, and a range of other therapeutic agents, her condition did not show sustained improvement. The patient was then administered atezolizumab, an antibody targeting programmed death ligand 1 (PD‐L1). Although initial results saw a disappearance of her skin tumors, the subsequent cycles led to symptoms consistent with stage IVA1 SS, coupled with an evident shift in malignant T‐cell clone dominance. Atezolizumab, a humanized monoclonal antibody, impedes the interaction of PD‐L1 with its receptor PD1, which is notably overexpressed in malignant T‐cells of SS. This case illustrates the emergence of SS during the course of atezolizumab treatment for a transformed MF condition, underlining the potential risks and complexities in using PD‐L1 inhibitors for treating CTCL.

Primary cutaneous lymphomas are a heterogeneous group of rare lymphoproliferative diseases that are initially limited to the skin but potentially also involve the lymph nodes, blood, and occasionally visceral organs. They develop from mature T cells, B cells, and NK cells. The classification includes a wide range of pathologies, each characterized by distinct clinical symptoms and variations in disease course. Cutaneous lymphomas are typically chronic in nature, and a complete cure is rarely achievable. Since primary cutaneous lymphomas are uncommon and present in many variants, they pose diagnostic and therapeutic challenges that require multidisciplinary collaboration between dermatologists, pathologists, hematologists, and oncologists. We present revised guidelines for the diagnosis and treatment of primary cutaneous lymphomas, with the goal of simplifying the diagnostic and therapeutic procedure in routine clinical practice. The guidelines cover the clinical, histopathological, and dermoscopic characteristics of skin lesions, along with recommendations for the diagnostic and therapeutic management of primary cutaneous lymphomas. When selecting the most suitable therapeutic approach for individual patients diagnosed with primary cutaneous lymphoma, the main factor to consider should be the clinical stage of the disease. However, other factors, such as the patient’s age, overall health, and medical history, should also be taken into account. The choice of therapy should be guided by finding optimal efficacy and safety. The guidelines also include globally recognized therapeutic approaches that are currently not accessible in Poland. The recommendations rely on the existing Polish and global guidelines, and the knowledge and experience of experts.

Abstract Background An increased risk of Secondary Malignancies (SMs) in Mycosis Fungoides (MF) has been suggested previously. However, the relationship between this risk and the features of MF is not well-known. Objective To investigate the rate and types of SMs in a large cohort of MF patients focusing on the associated features of these patients. Methods The demographic features, subtype, and stage of MF, as well as the temporal relationship between the diagnosis of MF and the development of SMs were determined. Major clinical features of MF in this group were compared with MF patients without association of SMs. Results Among 730 MF patients with a mean follow-up period of 67.9 ± 52.4 months, 56 SMs were identified in a total of 52 (7.1%) patients. While 28.8% of patients were previously diagnosed with other malignancies, then subsequently had a diagnosis of MF, it was vice versa in 53.8% of patients. Most of the SM-associated MF patients had early-stage (80.7%) and classical type of MF (86.5%) without a significant difference from MF patients without association of SMs; 85.5% and 72.5%, respectively. The most commonly identified SMs were hematologic malignancies (64.3%) including lymphomatoid papulosis (n = 22), Hodgkin’s lymphoma (n = 4), non-Hodgkin’s lymphoma (n = 5), polycythemia vera (n = 2). Other most commonly associated malignancies were breast cancer (n = 4), prostate cancer (n = 3), renal cell carcinoma (n = 2), melanoma (n = 2), and Kaposi’s sarcoma (n = 2). Study limitations A single tertiary dermatology center study with a retrospective design. Conclusion Apart from the well-known lymphomatoid papulosis association, systemic hematological malignancies were also quite common in the large cohort of MF patients.

Abstract Background Mycosis fungoides is the most frequent form of cutaneous T-cell lymphoma. It is characterized by a chronic, slow, and progressive course, and is associated with mortality rates that depend on several factors, such as clinical staging. A median survival time of up to 13 months is found in patients with advanced stages that require more aggressive treatments, with greater toxicity and higher costs. In Latin America, few prognostic studies of the disease are available. Objective To determine the rate of progression from early stages (IA, IB, IIA) to more advanced stages (> IIB) in patients older than 18 years with mycosis fungoides treated at two medical centers in Colombia between January 1, 2010, and December 31, 2019. Methods Retrospective cohort study with a longitudinal design. Results 112 patients diagnosed with early mycosis fungoides were included. 56.2% were male (n = 63), with a median age of 53 years (IQR 43‒67). The most frequent clinical variant was classic (67.9%; n = 76), followed by folliculotropic (16%; n = 18), and hypopigmented (10.7%; n = 12). The most common first-line treatment was NB-UVB phototherapy (27.7%; n = 31), followed by PUVA phototherapy (25.8%; n = 29%), and topical corticosteroids (25%; n = 28). The global rate of disease progression was 8% (n = 9), with an overall mortality of 12.5% (n = 14). Study limitations Its retrospective design and the lack of molecular studies for case characterization. Conclusions Early mycosis fungoides is a disease with a good prognosis in most patients, with a progression rate of 8% (n = 9).

Mycosis Fungoides (MF) is the most common cutaneous T-cell lymphoma that typically presents in the early phase as inflammatory erythematous patches or plaques, with epidermotropism as the histopathological hallmark of the disease. Traditionally, in the early stages, non-aggressive options represent the first-line strategy: topical corticosteroids, phototherapy, radiotherapy and occasionally adopting a 'wait-and-see' approach for minimally symptomatic patients. In patients with advanced or recurrence disease, good results can be achieved with immune modifiers, chemotherapeutic agents, total skin irradiation or extracorporeal photochemotherapy and maintenance therapy is often required. The past decade has seen an expansion of therapies that can be used in this setting by increasing new therapeutic strategies. Herein are resumed the key advancements coming from recently published trials.

The prognosis of patients with mycosis fungoides is variable. As the current literature is scarce and shows mixed results this study investigates the incidence of other primary malignancies in mycosis fungoides patients. A retrospective, nationwide, population- based cohort study was performed with patients with mycosis fungoides between 2000 and 2020 in The Netherlands. All histopathology reports were requested from the Nationwide Network and Registry of Histo- and Cytopathology and screened for other primary malignancies. Lifelong incidence rates were used to compare the incidence of malignancies in mycosis fungoides patients and the general population. In total 1,024 patients were included with a mean follow-up of 10 years (SD 6). A total of 294 cases of other primary malignancies were found with 29% of the mycosis fungoides patients developing at least 1 other primary malignancy. Only cutaneous (odds ratio [OR] 2.54; CI 2.0–3.2) and haematological malignancies (OR 2.62; CI 2.00–3.42) had a statistically significant higher incidence than the Dutch population overall. Mycosis fungoides patients have a significantly increased risk of developing melanomas (OR 2.76; CI 2.11–3.59) and cutaneous squamous cell carcinomas mycosis fungoides (OR 2.34; CI 1.58–3.45). This study shows no association between mycosis fungoides and other solid organ tumours; however, such patients are significantly at risk of developing other haematological and cutaneous malignancies. Clinicians should be aware of this increased risk.

Abstract Immunobiologicals represent an innovative therapeutic option in dermatology. They are indicated in severe and refractory cases of different diseases when there is contraindication, intolerance, or failure of conventional systemic therapy and in cases with significant impairment of patient quality of life. The main immunobiologicals used in dermatology basically include inhibitors of tumor necrosis factor-alpha (anti-TNF), inhibitors of interleukin-12 and -23 (anti-IL12/23), inhibitors of interleukin-17 and its receptor (anti-IL17), inhibitors of interleukin-23 (anti-IL23), rituximab (anti-CD20 antibody), dupilumab (anti-IL4/IL13) and intravenous immunoglobulin. Their immunomodulatory action may be associated with an increase in the risk of infections in the short and long term, and each case must be assessed individually, according to the risk inherent to the drug, the patient general condition, and the need for precautions. This article will discuss the main risks of infection associated with the use of immunobiologicals, addressing the risk in immunocompetent and immunosuppressed patients, vaccination, fungal infections, tuberculosis, leprosy, and viral hepatitis, and how to manage the patient in the most diverse scenarios.

Resumen: Antecedentes y objetivo: No se dispone de datos españoles sobre el coste asociado al linfoma cutáneo de células T (LCCT). Además, la incorporación de nuevos tratamientos hace necesario analizar el coste real de la enfermedad. El estudio MICADOS analizó dos objetivos principales: Por un lado, evaluó el impacto en la calidad de vida en los pacientes con LCCT, y por otro lado, estudió los costes de la enfermedad. En esta publicación se recoge el segundo de los objetivos del estudio. Métodos: El coste de la enfermedad se estudió bajo la perspectiva del Sistema Nacional de Salud (SNS) con un horizonte temporal de un año. Participaron 23 dermatólogos y hematólogos de 15 hospitales públicos españoles. Se incluyeron pacientes adultos con LCCT del tipo micosis fungoide (MF) y síndrome de Sézary (SS). Resultados: Se incluyeron 141 pacientes, el 57,4% masculinos, con una edad media de 63,6 años (IC 95%: 61,4-65,7). Los costes directos anuales medios por pacientes del estudio fueron de 34.214€, siendo de 11.952,47€ en estadio I, 23.506,21€ en estadio II, 38.771,81€ en estadio III y 72.748,84€ en estadio IV. El coste anual directo total estimado de todos los pacientes en España con MF/SS resultó en 78.301.171€, donde el 81% de los costes fueron atribuibles a pacientes en estadio I, el 7% al estadio II, el 6% al estadio III y el 6% al estadio IV. Conclusiones: Este estudio ofrece una evaluación precisa del coste directo del LCCT en pacientes con MF/SS en España, mostrando costes que varían sustancialmente en función del estadio. Los costes soportados por el paciente y los costes indirectos deberán considerarse en futuras investigaciones. Abstract: Background and objective: The cost of treating cutaneous T-cell lymphoma (CTCL) in Spain is unknown. With the advent of new treatments, it is more important than ever to gain an accurate picture of the true costs involved. The MICADOS study had 2 primary objectives: 1) to evaluate the impact of CTCL on patient quality of life, and 2) to evaluate the costs associated with the disease. This article reports the results of the cost analysis. Methods: We estimated the cost of treating CTCL over a period of 1 year from the perspective of the Spanish National Health System. Twenty-three dermatologists and hematologists from 15 public hospitals analyzed data for adult patients with mycosis fungoides (MF) or Sézary syndrome (SS). Results: A total of 141 patients (57.4% male) with a mean age of 63.6 years (95% CI: 61.4-65.7 years) were included. The mean direct annual cost of treating CTCL was €34,214 per patient. The corresponding costs by stage were €11,952.47 for stage I disease, €23,506.21 for stage II disease, €38,771.81 for stage III disease, and €72,748.84 for stage IV disease. The total direct annual cost of treating MF/SS in public hospitals in Spain was estimated at €78,301,171; stage I disease accounted for 81% of all costs, stage II for 7%, and stages III and IV for 6% each. Conclusions: The MICADOS study offers an accurate picture of the direct cost of treating CTCL in patients with MF/SS in Spain and shows that costs vary significantly according to disease stage. Patient-borne and indirect costs should be analyzed in future studies.

AbstractBackground: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma.Objectives: This study was conducted to evaluate efficacy and safety of interferon (IFN) α-2a combined with phototherapy for early-stage MF.Methods: Thirteen patients with early-stage MF received subcutaneous injections of IFN α-2a at 3 million IU combined with phototherapy three times per week for 6 months. Treatment efficacy was measured by changes in body surface area (BSA) score and modified severity-weighted assessment tool (mSWAT) score at 1, 3, and 6 months after treatment. Histopathologic examinations of skin lesions were performed before and after treatment.Results: After 3 months of treatment, all 13 patients achieved a partial response, and BSA and mSWAT scores were significantly lower than those at baseline (p

The most common and widespread type of cutaneous T-cell lymphoma is mycosis fungoides (MF), and it has a multiphasic clinical and biological course, with early stages being indolent for many years and later stages being faster and more aggressive. The clinical stage has a significant impact on the management and course of treatment: in the early stages, skin-directed therapies (SDT) plus/or biologic response modifiers (BRM); in the later stages, radiotherapy and/or systemic therapies. Even though national and international societies and groups periodically update their clinical recommendations, there is still no universally accepted approach. This paper reviews and discusses the various SDT and BRM options, either separately or in combination.

The most prevalent primary cutaneous T-cell lymphoma, mycosis fungoides (MF), is characterized by the development of plaques and nodules after an erythematous patchy phase that is non-specific. An infiltrate of atypical small- to medium-sized cerebriform lymphocytes in the superficial dermis, with variable epidermotropism, is the histopathological hallmark of the disease. In more advanced stages of the illness, large-cell transformation may be seen. Early diagnosis of MF can be very challenging based only on histopathologic or clinical findings, so it is critical to have a clinical-pathological correlation. Many atypical variants of MF that deviate from the classic Alibert-Bazin presentation of the disease have been described over the past 30 years, sometimes with different prognostic and therapeutic implications. Clinically or histopathologically, they can mimic a wide range of benign inflammatory skin disorders. To make a conclusive diagnosis in these cases, it is recommended to take multiple biopsies from various lesions and to carefully correlate the clinical and pathological findings. We have outlined the various facets of the illness in this review, positioning MF as a “great imitator”, with an emphasis on the more recently identified variations, differential diagnosis, and its benign mimics.

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most prevalent non-Hodgkin lymphomas that comprise cutaneous T-cell lymphomas (CTCL), accounting for more than 70% of cases. Following the Tumor Lymph nodes Metastasis Blood system, disease staging is carried out, and within ten years, about thirty percent of patients in the early stages will have advanced disease. Plaques, folliculotropism, and age over 60 are risk factors for progression. A 5-year survival rate of less than 20% is associated with LCT in MF. Treatment requires an interdisciplinary approach; skindirected therapies are available for early stages of the disease, but there are no curative options for advanced stages of the disease other than allogeneic stem cell transplantation. Because of their severe symptoms and poor treatment efficacy, patients in advanced stages have a lower quality of life and a lower chance of survival. In patients with CD30-expressing CTCL, Brentuximab Vedotin has demonstrated better response rates and progression-free survival (PFS); in advanced SS, mogamulizumab has significantly increased PFS. These findings emphasize the need to standardize prognostic factors and improve CTCL treatment.

Radiation therapy (RT) is administered with varying intentions, sometimes even several times in the same or in different body areas, to over 50% of patients with neoplastic conditions. Numerous techniques are available to patients in the clinical evolution of mycosis fungoides (MF), and there are several indications for radiation therapy (RT). RT as a skin-directed therapy is very widely used in these patients, either alone or in conjunction with other therapies. The application of RT, a tried-and-true therapy that improves MF patients’ quality of life and treatment, can be encouraged by a multidisciplinary approach and an understanding of current methods and action mechanisms.

This article discusses the case of a 9-year-old girl who presented with an enlarging ulcerated nodule on her left temple. The biopsy revealed a diagnosis of lymphomatoid papulosis (LyP), a CD30+ cutaneous T-cell lymphoproliferative disorder. LyP is characterized by recurrent crops of asymptomatic erythematous papulo-nodules that typically regress after 2-8 weeks. Treatment options for LyP include watchful waiting, topical or intralesional corticosteroids, surgical excision, radiation therapy, low-dose methotrexate, and narrowband ultraviolet B radiation phototherapy. Continued clinical monitoring is important to detect other lymphoproliferative disorders. [Extracted from the article]

Abstract Background Methyl‐aminolevulinate‐based photodynamic therapy (MAL‐PDT) has been successfully used in early‐stage mycosis fungoides (MF) lesions. However, the long‐term outcome of initially responding lesions has not been systematically investigated to date. To address this issue, we hereby report a large series of early‐stage MF lesions treated with MAL‐PDT and followed up for 1 year after initial treatment. Objectives The objective of this study was to retrospectively evaluate short‐ and middle‐term clinical results of MAL‐PDT on early‐stage MF lesions (patches and plaques) and to identify characteristics possibly predictive of effectiveness. Methods Data from all early‐stage MF lesions (plaques and patches) treated with MAL‐PDT between 2010 and 2017 in a tertiary referral centre were retrospectively collected. Primary endpoints were initial clinical response rated as complete response (CR) or partial response (PR), and the rate of relapse of initially responding lesions was further evaluated 1 year after the last PDT session. Secondary endpoints addressed tolerance and the relevancy of predefined parameters possibly predictive of CR achievement. Results A total of 62 lesions from 30 patients with early‐stage MF (21 Ia, nine Ib) were treated and analysed. At the 6‐week evaluation, the overall clinical response rate (RR) was 87.25% (55% CR and 32.25% PR) with a mean of 2.7 PDT sessions per lesion. Twenty‐five percent of initially responding lesions relapsed locally within 1 year. Transient, although significant, pain occurred at least once in 30% of patients. In univariate analysis, the location of lesions on sun‐protected areas was the only parameter statistically related to CR, a result confirmed in multivariate analysis. Conclusions Overall, these data confirm the interest in the use of MAL‐PDT in early‐stage MF even for deep (folliculotropic) lesions with a higher efficiency on sun‐protected areas. Furthermore, this study demonstrates for the first time that the response is often sustained over time. Accordingly, MAL‐PDT should be considered in the management of early‐stage MF, especially in oligolesional forms or in residual lesions refractory to systemic treatment.

Abstract Background Folliculotropic mycosis fungoides (FMF) is a rare variant of MF that involves hair follicles and requires a more aggressive treatment regime. Significantly, some patients with classic MF can eventually have some degree of follicular involvement. Objectives The aim of this study was to describe the dermoscopic features of FMF in nonscalp areas to facilitate early diagnosis and posttreatment monitoring. Methods The clinical and dermoscopic features in 11 FMF patients were analyzed and compared with the reports in the literature. Results Obliteration of hair follicles, loss of terminal hairs and hair loss were detected in all patients. Follicular accentuation, follicular dilation and broken hairs were found in almost all patients (90.9%). Other common follicular‐based dermoscopic features (

Mycosis Fungoides (MF) stands as the predominant form of primary cutaneous T-cell lymphoma (CTCL). It manifests a diverse array of clinical, histological, and immunophenotypic variations, each bearing distinct prognostic implications. The typical immunophenotypic profile of mycosis fungoides involves CD3+/CD4+/CD45RO+ memory T cells. Notably, the CD4-/CD8- double-negative variant of MF is a rare occurrence, observed in approximately 12% of early-stage cases and more prevalent in tumor-stage instances, often correlated with atypical clinical presentations. Despite its rarity, scant information is available about double-negative Mycosis Fungoides, with only a limited number of cases documented in the existing literature. This review aims to provide enhanced clarity, comprehension, and a detailed exploration of the spectrum encompassing double-negative mycosis fungoides.

Programmed cell death protein 1 (PD-1) plays a pivotal role in immune system regulation, with its expression levels linked to malignancy prognosis. However, existing reports on PD-1 staining in mycosis fungoides (MF) present conflicting findings, and little attention has been given to PD-1 staining in different MF variants. To address this, we conducted a retrospective study, employing immunohistochemistry to examine PD-1 expression in cases of folliculotropic MF and non-folliculotropic MF. We analyzed 24 cases of folliculotropic MF and 18 cases of non-folliculotropic MF, and recorded both the percentage of PD-1-labeled tumor cells and the intensity score (negative, weak, medium, or strong). Our results revealed significant disparity in PD-1 labeling between patch/plaque MF and folliculotropic MF (p = 0.028). Non-folliculotropic MF exhibited higher PD-1 labeling in tumor cells (58.3%) compared to folliculotropic MF (40.2%). Notably, there was no significant difference in PD-1 staining between folliculotropic MF and non-folliculotropic MF when both were in the early stage/indolent disease category. However, when considering the tumor stage, folliculotropic MF exhibited PD-1 staining in tumor cells at a rate of 21.1%, while non-folliculotropic MF showed PD-1 staining in tumor cells at a rate of 46.6% (p = 0.005). Additionally, among folliculotropic MF cases, 13 out of 24 cases displayed differing PD-1 expression patterns between epidermal and dermal components, with preserved PD-1 staining in the epidermal component and loss of staining in the dermal component. Furthermore, consistent with the prior literature, tumor cells with large cell transformations exhibited significantly lower PD-1 labeling (p = 0.017). Our findings showcase the unique PD-1 staining patterns in MF.

Mycosis fungoides (MF) and Sezary syndrome (SS) are important representatives of the heterogeneous group of cutaneous T-cell lymphoma. The diagnosis of MF/SS is based on the clinical, histopathologic and immunohistochemical findings, flow cytometric analysis of peripheral blood and T-cell receptor gene rearrangement, as well as other genetic analysis. The treatments for MF/SS are always stage-oriented and are classified into topical (skin-directed) and systemic therapies. Chinese and international experts in this field have updated recommendations and guidelines for the diagnosis and treatment of MF/SS. There are tremendous evolvements in the diagnosis of MF/SS, including molecular diagnostic methods, molecular biomarkers, dermoscopic imaging and artificial intelligence. Novel disease-specific targeted therapy, biologic therapy and immunomodulatory drugs have emerged. This review summarizes advances in the diagnosis and treatments of MF/SS, which may provide a reference for clinical practice and future research.

The large cell transformation of mycosis fungoides (MF-LCT) is a phenomenon observed in the advanced stages of mycosis fungoides (MF), which is the most common primary cutaneous lymphoma. The diagnostic criteria of MF-LCT are a minimum of 25% of large cells or a formation of microscopic nodules of them in the histological examination of skin samples. The clinical outcomes for MF-LCT are poor, as less than 20% of patients survive 5 years after diagnosis, but the expression of the CD30 antigen is generally considered to be associated with a better prognosis. We present a case of a patient with the diagnosis of MF with LCT, with an ulcerated tumor lesion approximately 30 × 20 cm in size on the right lateral abdominal wall. Brentuximab vedotin (BV) treatment was started due to the presence of the CD30 antigen, with a quick and impressive regression of the cutaneous lesion and tumor mass and good treatment tolerance. After follow-up of 20 months, patient remains in complete remission. A schedule of treatment for MF-LCT is directed mainly by the clinical stage of the disease and the comorbidities; the more severe clinical course of the disease requires systemic treatment. If at least 5% of the cells found in the skin lesions biopsy sample express the CD30 antigen, a beneficial effect of BV treatment could be expected. It may seem that the use of BV is one of the optimal therapeutic options in patients with advanced MF-LCT showing expression of CD30.

XiaoYan Zhang, Ping Wang Department of Dermatology, Hangzhou Third People’s Hospital, Hangzhou, People’s Republic of ChinaCorrespondence: Ping Wang, Department of Dermatology, Hangzhou Third People’s Hospital, Hangzhou, People’s Republic of China, Tel +86-13588812862, Email dermwang@aliyun.comAbstract: We present the case of a 37-year-old male diagnosed with Mycosis fungoides (MF) after gradually developing multiple skin tags and brownish lichenoid papules. The patient had pre-existing erythema over his entire body, especially his face, upper extremities, and trunk, for over 1.5 years. Microscopic examination of the papule and the skin tag (ST) exhibited similar features mainly characterized by superficial dense band-like lymphoid infiltrates and epidermotropism of atypical lymphocytes (Pautrier’s micro-abscesses). Immunohistochemistry further revealed the lymphoid infiltrates predominantly expressed LCA, CD3, CD4, and CD45RO but lacked CD7, CD8, CD30, CD20, and CD79a. The finding of this study that reports MF characterized by unusual STs suggests that some causes and effects have not been previously described in MF.Keywords: skin tags, lymphoma, T-Cell, cutaneous, mycosis fungoides