Identifying unmet needs and challenges in the definition of a plaque in mycosis fungoides: An EORTC-CLTG/ISCL survey.

BACKGROUND: Consensus about the definition and classification of 'plaque' in mycosis fungoides is lacking. OBJECTIVES: To delineate a comprehensive view on how the 'plaque' entity is defined and managed in clinical practice; to evaluate whether the current positioning of plaques in the TNMB classification is adequate. METHODS: A 12-item survey was circulated within a selected panel of 22 experts (pathologists, dermatologists, haematologists and oncologists), members of the EORTC and International Society for Cutaneous Lymphoma. The questionnaire discussed clinical and histopathological definitions of plaques and its relationship with staging and treatment. RESULTS: Total consensus and very high agreement rates were reached in 33.3% of questions, as all panellists regularly check for the presence of plaques, agree to evaluate the presence of plaques as a potential separate T class, and concur on the important distinction between plaque and patch for the management of early-stage MF. High agreement was reached in 41.7% of questions, since more than 50% of the responders use Olsen's definition of plaque, recommend the distinction between thin/thick plaques, and agree on performing a biopsy on the most infiltrated/indurated lesion. High divergence rates (25%) were reported regarding the possibility of a clinically based distinction between thin and thick plaques and the role of histopathology to plaque definition. CONCLUSIONS: The definition of 'plaque' is commonly perceived as a clinical entity and its integration with histopathological features is generally reserved to specific cases. To date, no consensus is achieved as for the exact definition of thin and thick plaques and current positioning of plaques within the TNMB system is considered clinically inadequate. Prospective studies evaluating the role of histopathological parameters and other biomarkers, as well as promising diagnostic tools, such as US/RM imaging and high-throughput blood sequencing, are much needed to fully integrate current clinical definitions with more objective parameters.

Extracorporeal photopheresis and multimodality therapy in patients with T-cell cutaneous lymphomas: Real-life experience in Argentina.

BACKGROUND: Extracorporeal photopheresis (ECP) as a part of multimodality therapy, is one of the treatments for Sézary syndrome (SS) and advanced stage mycosis fungoides (MF). This study aims to describe cutaneous and peripheral blood responses of patients with MF and SS who received multimodality therapy. METHODS: In this cross-sectional retrospective study, patients with MF or SS who received ECP treatment in combination with at least one additional systemic treatment between 2011 and 2018 were included. ECP consisted of a two-session cycle every 2 to 4 weeks. Cutaneous and blood responses were evaluated with updated criteria. RESULTS: Twenty-eight patients (11 (39%) with MF and 17 (51%) with SS) were included. Their median age at diagnosis was 63 (57-67) years. The median number of treatments before ECP was 2 (1-3). Seven out of 11 patients with MF (63%) underwent an assessment of cutaneous response. Five patients (70%) presented a partial response; 1 (15%), stable disease, and 1 (15%) progressive disease. Thirteen of the 17 patients with SS (76%) underwent evaluation. One patient (8%) presented a complete cutaneous response; 6 (46%), a partial response; 5 (38%), stable disease; and 1 (8%), progressive disease. None of them relapsed during the study period in both groups. No ECP-related adverse effects occurred during the study. CONCLUSION: Most patients with SS and MF who underwent multimodality therapy with ECP had favorable cutaneous and blood response. It is safe to combine ECP with other treatments. Studies with large numbers of patients are necessary to assess the effects of ECP on patient survival.

Primary cutaneous lymphoma in Argentina: a report of a nationwide study of 416 patients.

BACKGROUND: The aim of this study was to determine the relative frequency of primary cutaneous lymphoma (PCL) in Argentina according to the new World Health Organization (WHO)-European Organization for the Research and Treatment of Cancer (EORTC) classification system. METHODS: A total of 416 patients from 21 dermatology services were included during a 5-year period (2010-2015); these patients were classified using WHO-EORTC criteria. RESULTS: There were 231 (55.2%) males and 185 (44.8%) females; the male-to-female ratio was 1.35. The median age of the patients was 57 years (range, 0-90 years). Most patients were Caucasian (79%), and only 16% of patients were registered as Amerindian. Most patients (387/416, 93%) had cutaneous T-cell lymphoma (CTCL); 28 patients (6.7%) were diagnosed with cutaneous B-cell lymphoma (CBCL). The most frequent CTCL subtypes, in decreasing order of prevalence, were mycosis fungoides (MF), including its variants (75.7%); CD30+ primary cutaneous lymphoproliferative disorders (7.2%); and Sézary syndrome (SS) (3.1%). Cutaneous follicle center lymphoma was the most common CBCL subtype (2.9%). In the subset of patients ≤20 years of age, the most common condition was MF (57%), followed by extranodal NK-T nasal-type lymphoma (14%). CONCLUSIONS: This study revealed relatively higher rates of MF and lower rates of CBCL in Argentinean patients that have been reported in American and European countries.

No detection of c-kit gene mutations in exons 9, 11, 13 and 17 and low CD117 expression in plaque-stage mycosis fungoides

Abstract: The growth factor receptor c-kit (CD117) is expressed in immature T-cells and in some advanced forms of mycosis fungoides. c-kit gene mutation results in unrestricted neoplastic proliferation. We aimed to detect by PCR the most frequent exon mutations in seventeen plaque-stage MF patients, in their perilesional skin and in healthy skin donors. We secondarily evaluated CD117 expression by immunohistochemistry in plaque-stage and tumor-stage MF. We detected no mutation in c-kit gene and low CD117 expression was confirmed on atypical cells in one patient. Complete c-kit exon and intron sequences should be assessed and more sensitive sequencing method could be also applied.

No detection of c-kit gene mutations in exons 9, 11, 13 and 17 and low CD117 expression in plaque-stage mycosis fungoides.

The growth factor receptor c-kit (CD117) is expressed in immature T-cells and in some advanced forms of mycosis fungoides. c-kit gene mutation results in unrestricted neoplastic proliferation. We aimed to detect by PCR the most frequent exon mutations in seventeen plaque-stage MF patients, in their perilesional skin and in healthy skin donors. We secondarily evaluated CD117 expression by immunohistochemistry in plaque-stage and tumor-stage MF. We detected no mutation in c-kit gene and low CD117 expression was confirmed on atypical cells in one patient. Complete c-kit exon and intron sequences should be assessed and more sensitive sequencing method could be also applied.

Importancia del seguimiento de lesiones melanocíticas con dermatoscopia digital para el diagnóstico temprano de melanoma / Importance of monitoring melanocytic lesions with digital dermatoscopy for diagnosis early melanoma

La dermatoscopia digital es una herramienta que permite el diagnóstico de melanomas en estadios tempranos, por medio del seguimiento de las lesiones pigmentarias a largo plazo. Se comunican tres casos de pacientes con alto riesgo de melanoma, en los cuales ‒a través del seguimiento con dermatoscopia digital‒ se realizó el diagnóstico de la enfermedad mediante la detección de cambios morfológicos, arquitecturales y de pigmentación de las lesiones estudiadas. (AU)

Digital dermoscopy is a tool that allows the early diagnosis of melanomas, through the long-term follow up of pigmentary skin lesions. We report three cases of patients with high-risk of melanoma, in which the diagnosis had been made by morphological, arquitectural and pigmentary changes observed by the digital dermoscopy follow-up. (AU)

Llega la primavera y florecen los eritemas polimorfos solares / Spring arrives and with it the polymorphous light eruption

El eritema polimorfo solar es la fotodermatosis más frecuente y suele aparecer en primavera con la primera exposición intensa al sol. Sus manifestaciones cutáneas son variadas y el diagnóstico se basa en la clínica junto al antecedente de exposición solar. En los casos leves, la fotoprotección suele ser suficiente para el control de la enfermedad, pero en formas más graves se requieren otras terapéuticas, como corticoides, antihistamínicos, o fototerapia, que genera una "fotoadaptación" de las áreas de piel afectadas. Presentamos un caso típico de erupción polimorfa solar que respondió de forma adecuada a medidas de fotoprotección. (AU)

The polymorphic solar eruption is the most frequent photodermatosis, and usually appears in spring with the first intense exposure to the sun. It has multiple cutaneous manifestations, and its diagnosis is based on the clinic and the antecedent of solar exposition. In mild cases, photoprotection is usually enough to control the disease, but in more severe forms, other therapies are required, such as corticosteroids, antihistamines, or phototherapy to generate a "photo-adaptation" of the affected skin areas. We present a typical case of polymorphic solar eruption that responded adequately to photoprotection measurements. (AU)

Serie parasitosis en dermatología: tungiasis / Dermatology iconography: tungiasis

La tungiasis es una ectoparasitosis causada por la penetración en la piel de la pulga hembra Tunga penetrans. Esta enfermedad no es específica del hombre y se distribuye en regiones cálidas y secas alrededor del mundo. Se adquiere por contacto directo con el suelo en donde habitan los parásitos adultos. En zonas endémicas, su alta prevalencia se asocia a pobreza y falta de acceso al sistema de salud. Además es frecuente entre viajeros que visitan regiones endémicas y que regresan a sus lugares de origen. Presentamos un caso típico de tungiasis en un paciente 39 años que había realizado un viaje reciente a zona endémica. (AU)

Tungiasis is an ectoparasitosis caused by penetration into the skin of the female flea Tunga penetrans. This disease is not human-specific and is distributed worldwide in warm, dry regions. It is acquired by direct contact with the soil where the adult parasites live. In endemic areas, its high prevalence is associated with poverty and lack of access to the health system. It is also frequent among travelers visiting endemic regions and returning to their places of origin. We present a typical case of tungiasis in a 39 year old patient who had made a recent trip to an endemic area. (AU)

Desafíos terapéuticos en la enfermedad injerto contra huésped / Therapeutic challenges in graft versus host disease

La enfermedad injerto contra huésped es una entidad en la cual las células inmunológicas competentes de un tejido injertado reconocen y dañan antígenos presentes en el receptor del trasplante, que es incapaz de defenderse de ellas. Es una complicación frecuente del trasplante alogénico de médula ósea, y con menor frecuencia se produce luego de trasplantes de órganos sólidos o transfusiones de hemoderivados no irradiados. Se comunica el caso de una paciente de sexo femenino de 23 años, con leucemia linfoblástica aguda.y trasplante alogénico de médula ósea, que presentó una enfermedad injerto contra huésped con compromiso cutáneo y gastrointestinal dependiente de corticoides, con mejoría de los signos y síntomas cutáneos luego del tratamiento con infliximab y fotoféresis extracorpórea. (AU)

Graft versus host disease is an entity in which competent grafted immune cells recognize and damage tissue antigens present in the transplant recipient, who is unable to defend from them. It is one of the most serious complications in patients undergoing allogeneic bone marrow transplantation, although less frequently it may be associated with solid organ transplants or transfusions of not irradiated blood products. We report the case of a 23 year-old patient with acute lymphoblastic leukemia and allogeneic bone marrow transplantation, that presented graft versus host disease with skin and gastrointestinal involvement, dependent on corticosteroids, that showed improvement in signs and skin symptoms after treatment with infliximab and extracorporeal photopheresis. (AU)

Serie parasitosis en dermatología. Miasis: diferentes formas de presentación clínica / Parasitic series in dermatology. Myiasis: different forms of clinical presentation

La miasis es una infestación por larvas en desarrollo de una gran variedad de moscas del orden Díptera. Según el sitio de invasión, se clasifica en miasis cavitarias, que pueden deberse a invasión de cavidades naturales o de heridas, y miasis forunculoide, cuando atraviesa piel indemne. Esta infestación presenta una distribución mundial, con variaciones estacionales en relación con la latitud geográfica y el ciclo de vida de distintas especies de moscas. Presentamos una serie de tres casos de pacientes con distintas formas clínicas de manifestación de miasis. (AU)

Myiasis is the tissue infestation by a variety of Diptera order larvae flies. According to the invasion site, they are classified in myiasis of cavities, which can be because of an invasion of natural cavities or wounds, and furuncular myiasis, when they invade through intact skin. This infestation has a worldwide distribution, with seasonal variations in relation to the geographic latitude and the life cycle of different species of flies. We present three cases of patients with different clinical forms of presentation of myiasis. (AU)

Síndrome de Laugier-Hunziker / Laugier-Hunziker syndrome

El síndrome de Laugier-Hunziker es un trastorno pigmentario adquirido poco frecuente, caracterizado por presentar lesiones hiperpigmentadas cutaneomucosas idiopáticas que pueden asociarse a melanoniquia longitudinal. A pesar de ser considerado una enfermedad benigna sin manifestaciones sistémicas ni potencial maligno, es clave realizar el diagnóstico diferencial con otros trastornos pigmentarios, en especial con el síndrome de Peutz-Jeghers. Se presenta aquí el caso de un paciente con este síndrome poco frecuente. (AU)

Laugier-Hunziker syndrome is a rare acquired pigmentary disorder that is characterized by idiopathic mucocutaneous pigmentation that may be associated with longitudinal melanonychia. Although this syndrome is considered a benign disease with no systemic manifestations or malignant potential, it is important to rule out other mucocutaneous pigmentary disorders, especially Peutz-Jeghers syndrome. We report the case of a patient with this unusual syndrome. (AU)

Arte y naturaleza humana XXV / Art and human nature XXV

La malevolencia consiste en la realización de actos que dañan, en forma grave y éticamente inexcusable, el bienestar del otro. Tres factores son los que propician su aparición: el egoísmo excesivo, la falta de empatía y la debilidad interior. La obra Las ciudades invisibles de Italo Calvino constituye una buena oportunidad para reflexionar sobre la malevolencia y cómo esta puede ser neutralizada. (AU)

Malevolence consists of performing acts that damage in severe and ethically inexcusable manner, the welfare of the other. Three factors can induce its appearance: excessive selfishness, lack of empathy and inner weakness. The work The invisible cities by Italo Calvino is a good opportunity to reflect on the malevolence and how it can be neutralized. (AU)