Experience With Bexarotene to Treat Cutaneous T-Cell Lymphomas: A Study of the Spanish Working Group of Cutaneous Lymphomas.

BACKGROUND AND OBJECTIVES: Bexarotene has been approved to treat advanced stage cutaneous T-cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL. MATERIAL AND METHOD: This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or Sézary syndrome (42) on a 10-year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals. RESULTS: A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27-95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1-114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment-related grade 5 adverse events were reported. CONCLUSIONS: Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL.

Experience With Bexarotene to Treat Cutaneous T-Cell Lymphomas: A Study of the Spanish Working Group of Cutaneous Lymphomas. / [Artículo traducido] Experiencia con bexaroteno en linfoma cutáneo de células T: un estudio del Grupo Español de Linfomas Cutáneos (GELC).

BACKGROUND AND OBJECTIVES: Bexarotene has been approved to treat advanced stage cutaneous T-cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL. MATERIAL AND METHOD: This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or Sézary syndrome (42) on a 10-year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals. RESULTS: A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27-95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1-114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment-related grade 5 adverse events were reported. CONCLUSIONS: Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL.

Blastic Plasmacytoid Dendritic Cell Neoplasm: A Single-Center Experience. Clinical Characterization, Mutational Landscape, and Clinical Outcome of Patients Undergoing Hematopoietic Stem Cell Transplantation Intensive Therapy.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematodermic neoplasm usually involving the skin. In this retrospective case series, 10 cases of BPDCN were identified, 90% of which had skin involvement and exhibited predominantly violaceous nodules and/or bruise-like plaques. Skin lesions showed diffuse or nodular dermal-based infiltrates of intermediate sized blasts with a grenz zone. Tumor immunophenotyping was CD4(+), CD56(+), CD123(+) and CD303(+). The most frequently mutated genes according to targeted next-generation sequencing were TET2 (3/7) and NRAS (2/7). Multiagent chemotherapy (CT) was administered as first-line therapy, and a total of 5 patients underwent allogenic hematopoietic stem cell transplantation (allo-HSCT). Better outcomes were observed in younger patients and those treated with acute lymphoblastic leukemia (ALL)-like CT followed by allo-HSCT. This study shows the clinical range of cutaneous lesions of BPDCN. Despite the absence of a gold standard therapy, patients treated with myeloablative intensive regimens and allo-HSCT seems to have a more favorable prognosis.

Clinical variants of mycosis fungoides in a cohort.

INTRODUCTION: Mycosis fungoides (MF) is the most common primary skin T-cell lymphoma, which is characterized for a heterogeneous clinical expressivity. OBJECTIVE: To report clinical variants and sociodemographic characteristics in patients with MF under the care of a dermatological hospital. METHODS: 290 patients with MF clinical and histopathological diagnosis attended to over the course of 11 years were included. Sociodemographic description of patients was made, who were classified according to clinical and histopathological variants. RESULTS: MF was recorded in 57.9 % of women and 42 % of men. The most common clinical variant was the classic type in 46.2 %; dyschromic variants accounted for 35.2 %, out of which hypopigmented MF was the most representative (17.6 %); poikilodermatous MF accounted for 4.1 %, and folliculotropic, for 3.1%. The papular variant occurred in six patients (2.1 %), the single-plaque variety in three (1%), and the ichthyosiform, syringotropic and granulomatous slack skin varieties occurred in one patient each. The granulomatous variant was found in 0.7 %, and 1.4 % had erythroderma. CONCLUSIONS: The most common MF clinical variant was classic plaque stage, followed by dyschromic variants. Other clinical variants accounted for 18.6 %.

INTRODUCCIÓN: La micosis fungoide es el linfoma primario de células T en piel más frecuente, con expresividad clínica heterogénea. OBJETIVO: Reportar las variedades clínicas y las características sociodemográficas en pacientes con micosis fungoide tratados en un hospital dermatológico. MÉTODOS: Se incluyeron 290 pacientes con diagnóstico clínico e histopatológico de micosis fungoide atendidos en el transcurso de 11 años. Se realizó descripción sociodemográfica de los pacientes, quienes se clasificaron conforme las variantes clínicas e histopatológicas. RESULTADOS: La micosis fungoide se presentó en 57.9 % mujeres y 42 % hombres. La variedad clínica más común fue la clásica en 46.2 %; la discrómica representó 35.2 %, del cual la hipopigmentada fue la más representativa (7.6 %); la poiquilodérmica constituyó 4.1 % y la foliculotrópica, 3.1 %. La variedad papular se presentó en seis pacientes (2.1 %), la de placa única en tres (1 %) y la ictiosiforme, siringotrópica y la piel laxa granulomatosa, en un paciente cada una. La variedad granulomatosa se encontró en 0.7 % y 1.4 % presentó eritrodermia. CONCLUSIONES: La variedad clínica más frecuente de micosis fungoide fue la clásica en fase de placa, seguida de las variedades discrómicas. Otras variedades clínicas representaron 18.6 %.

Linfoma anaplásico de células T grandes primario cutáneo CD30+. Serie de nueve casos.

INTRODUCTION: CD30+ anaplastic large T cell lymphoma is a cutaneous primary lymphoma in which there is no evidence of systemic disease; histopathological study is required for its diagnosis. OBJECTIVE: To present the cases diagnosed with primary cutaneous CD30+ anaplastic large T-cell lymphoma over a 24-year period in Hospital General "Dr. Manuel Gea González" Department of Dermatology. METHOD: Retrospective study. Descriptive statistics was carried out. Information was collected on gender, age, clinical characteristics, complementary test results, previous and current treatments, histopathological studies reports and immunohistochemistry test results. RESULTS: Of 29 309 records, nine patients (0.000034%) with a diagnosis of CD30+ anaplastic T cell lymphoma were found. Histopathological and immunohistochemical diagnosis was confirmed by two certified dermatopathologists. Average age was 61.2 years, and there was a predominance of the female gender, with initial clinical presentation as a papular or nodular lesion and varied topography. CONCLUSIONS: The prognosis of CD30+ anaplastic large T cell lymphoma in the studied population was dependent on clinical stage. The treatment at early stages has favorable results.

INTRODUCCIÓN: El linfoma anaplásico de células T grandes CD30+ es un linfoma primario cutáneo en el cual no hay evidencia de enfermedad sistémica; para su diagnóstico es necesario el estudio histopatológico. OBJETIVO: Presentar los casos diagnosticados en el Departamento de Dermatología del Hospital General "Dr. Manuel Gea González" con linfomas anaplásicos de células T grandes primarios cutáneos CD30+ durante un periodo de 24 años. MÉTODO: Estudio retrospectivo en el que realizó estadística descriptiva. Se recopiló información de sexo, edad, características clínicas, resultados de pruebas complementarias, tratamientos previos y actuales, reportes de los estudios histopatológicos y de inmunohistoquímica. RESULTADOS: Entre 29 309 expedientes, se encontraron nueve casos (0.000034 %) con diagnóstico de linfoma anaplásico de células T CD30+. Se hizo la confirmación del diagnóstico histopatológico e inmunohistoquímico por dos dermatopatólogos certificados. La edad promedio fue de 61.2 años, hubo predominio del sexo femenino y de lesión papular o nodular y topografía variada como presentación clínica inicial. CONCLUSIONES: El pronóstico del linfoma anaplásico de células T grandes CD30+ en la población estudiada fue dependiente del estadio clínico. El tratamiento en etapas tempranas tiene resultados favorables.

Mycosis Fungoides: Experience in a Pediatric Hospital. / Micosis fungoide. Experiencia en un hospital pediátrico.

Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. OBJECTIVES: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. MATERIAL AND METHOD: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. RESULTS: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. CONCLUSIONS: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary.

Spontaneous regression of primary diffuse large B-cell lymphoma, leg type.

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT) accounts for approximately 20% of all primary cutaneous B-cell lymphomas and tends to present as infiltrated nodules, tumors, and plaques on the legs in the elderly. Unlike other primary cutaneous large B-cell lymphomas, it has a poor prognosis and tends to require treatment with systemic chemotherapy. We present the case of an 82-year-old patient with a 1-year history of nodules and plaques on her right leg. Biopsy led to a diagnosis of PCLBCL LT and the lesions resolved without treatment within 1 month of the first visit. This is an atypical course of PCLBCL LT and we believe that it is the first such case to be reported in the literature.

Rituximab in the treatment of primary cutaneous B-cell lymphoma: a review.

Rituximab is a chimeric mouse-human antibody that targets the CD20 antigen, which is found in both normal and neoplastic B cells. In recent years, it has been increasingly used to treat cutaneous B-cell lymphoma and is now considered an alternative to classic treatment (radiotherapy and surgery) of 2 types of indolent lymphoma, namely, primary cutaneous follicle center lymphoma and primary cutaneous marginal zone B-cell lymphoma. Rituximab is also administered as an alternative to polychemotherapy in the treatment of primary cutaneous large B-cell lymphoma, leg type. Its use as an alternative drug led to it being administered intralesionally, with beneficial effects. In the present article, we review the literature published on the use of rituximab to treat primary cutaneous B-cell lymphoma.

Blistering and aggressive paniculite cutaneous T-cell lymphoma.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is characterized by atypical T-cells expressing the α/ß T-cell receptor in the subcutaneous fat. Although it is usually indolent, some cases can show an aggressive course. It is usually a disease of the middle-aged, but can rarely affect children. CASE REPORT: We describe the case of a 12-year-old male, previously healthy, who presented a dermatosis disseminated to the four segments consisting of vesicles, blisters, erythematous and hematonecrotic plaques, atrophic scars, associated with edema. The biopsy confirmed limited cutaneous panniculitic T-cell lymphoma with extensive epidermal necrosis. CONCLUSIONS: We report the case of a SPTCL in a child. Although rare in this age group, the diagnosis should be considered in children who present similar conditions and who do not respond to treatment. Diagnosis is made on clinical suspicion and confirmed by histology. We discuss the challenges in its management and how timely diagnosis influences patient survival.

INTRODUCCIÓN: El linfoma de células T tipo paniculitis subcutánea (LCCTP) se caracteriza por la presencia de linfocitos T atípicos que expresan el receptor de células T α/ß en el tejido celular subcutáneo. Aunque generalmente es indolente, algunos casos presentan un curso agresivo. Es mayormente una enfermedad de la mediana edad, rara vez afecta a los niños. CASO CLÍNICO: Se describe el caso de un paciente de sexo masculino de 12 años de edad, previamente sano que presentó una dermatosis diseminada a los cuatro segmentos constituida por vesículas, ampollas, placas eritematocostrosas y hematonecróticas, además de atróficas, asociadas con edema. La biopsia confirmó linfoma cutáneo de células T paniculítico con extensa necrosis epidérmica. CONCLUSIONES: Reportamos el caso de un LCCTP en un paciente pediátrico. Aunque es raro en este grupo de edad, se debe considerar en los niños que presentan cuadros similares y que no responden a tratamiento. El diagnóstico se realiza por sospecha clínica y se confirma por histología. Se discuten los desafíos en su manejo y cómo el diagnóstico oportuno influye en la sobrevida del paciente.

[Papular mycosis fungoides in elderly: Report of two cases]. / Micosis fungoide papular en el anciano: a propósito de 2 casos.

Papular mycosis fungoides (MF) is an uncommon clinical variant of early MF without prognostic implications that follows an indolent course over years. It is characterized by the presence of multiple, small, pruritic, flat-topped, erythematous papules, often presenting as a nonspecific papular eruption which makes early diagnosis difficult. We describe two cases of elderly patients with papular MF, a probably underdiagnosed entity, which causes a significant deterioration in quality of life of patients who may benefit from specific treatments such as phototherapy.

Primary Cutaneous Acral CD8+ T-cell Lymphoma Induced by Persistent Delayed Hypersensitivity to Gold Earrings. / Linfoma cutáneo primario T CD8+ tipo acral inducido por hipersensibilidad retardada persistente a pendientes de oro.

Primary cutaneous CD8+ T-cell lymphoma has been included as a provisional entity within the new revised classification of lymphoid neoplasms of the World Health Organization in 20161. It was initially described as indolent CD8+ lymphoid proliferation of the ear2 and a total of 29 cases of such neoplasm have been published in the literature so far. None of them have been linked to delayed contact hypersensitivity reactions. We present a case of acral type primary cutaneous lymphoma T CD8+ involving both earlobes clearly related with the prolonged use of gold earrings, confirmed with epicutaneous tests, histopathology, immunohistochemical and molecular studies. Auricular skin lesions were induced again with a provocation test with identical histopathologycal and the same clonality, confirming both the diagnosis of lymphoma and its induction by the antigenic stimulus of gold.

Mycosis Fungoides in Children and Adolescents: A Series of 23 Cases. / Micosis fungoide en niños y adolescentes: descripción de una serie de 23 casos.

BACKGROUND: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma in adults and children. The prevalence has increased in some countries, but no descriptive studies of MF in the pediatric population have been done in Colombia to date. METHODS: A combined prospective-retrospective study of 128 patients with a diagnosis of MF confirmed by the dermatology department and dermatopathology laboratory of Universidad de Antioquia between 2008 and 2017. We describe the clinical and histopathologic variants, response to treatment, and progression of the disease in 23 patients under 18 years of age. RESULTS: The pediatric cases of MF accounted for 18% of all the cases on record. The median age of onset of lesions was 9 years, the median age at diagnosis was 11 years, and the median time between onset of lesions and diagnosis was 2 years. All patients were in early stages of the disease. Hypopigmented MF was the most common clinical presentation (in 52.2%), followed by classical MF (in 30.4%). Folliculotropic MF was identified in 17.4%. All patients were treated with topical corticosteroids and phototherapy. One patient received chemotherapy while still in the early stage of disease. Complete remission was achieved in 59.1% and a partial response in 40.9%. Only 2 patients remained asymptomatic for 5 years. CONCLUSION: We found hypopigmented MF to be the most common clinical presentation in patients under 18 years of age. The disease did not progress to advanced stages in any of the patients, although recurrence after treatment interruption was common.

Linfoma cutáneo de células T paniculítico, ampolloso y agresivo / Blistering and aggressive paniculite cutaneous T-cell lymphoma

Resumen Introducción: El linfoma de células T tipo paniculitis subcutánea (LCCTP) se caracteriza por la presencia de linfocitos T atípicos que expresan el receptor de células T α/β en el tejido celular subcutáneo. Aunque generalmente es indolente, algunos casos presentan un curso agresivo. Es mayormente una enfermedad de la mediana edad, rara vez afecta a los niños. Caso clínico: Se describe el caso de un paciente de sexo masculino de 12 años de edad, previamente sano que presentó una dermatosis diseminada a los cuatro segmentos constituida por vesículas, ampollas, placas eritematocostrosas y hematonecróticas, además de atróficas, asociadas con edema. La biopsia confirmó linfoma cutáneo de células T paniculítico con extensa necrosis epidérmica. Conclusiones: Reportamos el caso de un LCCTP en un paciente pediátrico. Aunque es raro en este grupo de edad, se debe considerar en los niños que presentan cuadros similares y que no responden a tratamiento. El diagnóstico se realiza por sospecha clínica y se confirma por histología. Se discuten los desafíos en su manejo y cómo el diagnóstico oportuno influye en la sobrevida del paciente.

Abstract Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is characterized by atypical T-cells expressing the α/β T-cell receptor in the subcutaneous fat. Although it is usually indolent, some cases can show an aggressive course. It is usually a disease of the middle-aged, but can rarely affect children. Case report: We describe the case of a 12-year-old male, previously healthy, who presented a dermatosis disseminated to the four segments consisting of vesicles, blisters, erythematous and hematonecrotic plaques, atrophic scars, associated with edema. The biopsy confirmed limited cutaneous panniculitic T-cell lymphoma with extensive epidermal necrosis. Conclusions: We report the case of a SPTCL in a child. Although rare in this age group, the diagnosis should be considered in children who present similar conditions and who do not respond to treatment. Diagnosis is made on clinical suspicion and confirmed by histology. We discuss the challenges in its management and how timely diagnosis influences patient survival.

The First Year of the AEVD Primary Cutaneous Lymphoma Registry. / Registro de linfomas cutáneos primarios de la AEDV: primer año de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. PATIENTS AND METHODS: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. RESULTS: In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ T-cell lymphoproliferative disorder (CD30+ CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30+ CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). CONCLUSION: The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group.

[Primary cutaneous B-cell lymphomas: study of 22 cases]. / Linfomas B cutáneos primarios: estudio de 22 casos.

INTRODUCTION AND OBJECTIVES: Primary cutaneous B-cell lymphoma (CBCL) is a very low prevalence neoplasm and constitutes 25% of all primary cutaneous lymphomas. Our objective was to discover the epidemiological, clinic and histologic characteristics of CBCL in our area. METHODS: Retrospective descriptive study with patients with histologic diagnosis of CBCL followed up in our department between 2004 and 2015. RESULTS: Twenty-two patients with CBCL were included; 65% were men and 35% were women. Follicle centre lymphoma was the most common subtype (41%). Only 3 cases presented with node involvement and one with bone marrow invasion. Five recurrences were detected and one patient died because of the CBCL. DISCUSSION AND CONCLUSIONS: This is one of the first CBCL series in theSpanish population. The incidence, sex, age, subtype distribution, clinical features and immunohistochemical patterns are very similar to those of the other series.

[Thyroid and lipidic dysfunction associated with bexarotene in cutaneous T-cell lymphoma]. / Disfunción tiroidea y lipídica asociada a bexaroteno en el linfoma cutáneo de células T.

BACKGROUND AND OBJECTIVE: Bexarotene is a synthetic selective X receptor rexinoide approved for the systemic treatment of cutaneous T-cell lymphoma. During treatment is very frequent the occurrence of hypothyroidism and severe mixed hyperlipidemia, both are reversibles and dose-dependent adverse events. Increase of triglycerides and LDL-cholesterol level (up to even higher levels have been associated with pancreatitis in some cases) is widely described (as is the case with other retinoids) but decrease in HDL-cholesterol is poored know. We review our experience with the use of bexarotene. MATERIAL AND METHODS: We present a serie of 3 clinical report of patients treated with bexarotene in whose, in addition to these well-known adverse event, a serious lowering of HDL-cholesterol was observed. RESULTS: The 3 patients studied had metabolic complications like central hypothyroidism and severe mixed hyperlipidemia; with special emphasis on the sharp fall (mean decrease>83%) in the HDL-cholesterol level. Cholesterol lowering medication and substitutive hormonal replacement with levotiroxine resulted in an improvement of the biochimical parameters without reaching the correct goals. CONCLUSIONS: Bexarotene produce as predictable side effects severe mixed hyperlipidemia with marked decrease in HDL-cholesterol levels and central hypothyroidism, being the both reversible and dose-dependent. A reflection on the possible aetiological mechanisms and implications of this phenomenon are included.

Variedades clínicas de micosis fungoide en una cohorte / Clinical variants of mycosis fungoides in a cohort

Resumen Introducción: La micosis fungoide es el linfoma primario de células T en piel más frecuente, con expresividad clínica heterogénea. Objetivo: Reportar las variedades clínicas y las características sociodemográficas de pacientes con micosis fungoide tratados en un hospital dermatológico. Métodos: Se incluyeron 290 pacientes con diagnóstico clínico e histopatológico de micosis fungoide atendidos en el transcurso de 11 años. Se realizó descripción sociodemográfica de los pacientes, quienes se clasificaron conforme las variantes clínicas e histopatológicas. Resultados: 58 % de los casos de micosis fungoide se presentó en mujeres y 42 % en hombres. La variedad clínica más común fue la clásica en 46.2 %; la discrómica representó 35.2 %, del cual la hipopigmentada fue la más representativa (7.6 %); la poiquilodérmica constituyó 4.1 % y la foliculotrópica, 3.1 %. La variedad papular se presentó en seis pacientes (2.1 %), la de placa única en tres (1 %) y la ictiosiforme, siringotrópica y la piel laxa granulomatosa, en un paciente cada una. La variedad granulomatosa se encontró en 0.7 % y 1.4 % presentó eritrodermia. Conclusiones: La variedad clínica más frecuente de micosis fungoide fue la clásica en fase de placa, seguida de las variedades discrómicas. Otras variedades clínicas representaron 18.6 %.

Abstract Introduction: Mycosis fungoides (MF) is the most common primary skin T-cell lymphoma, which is characterized for a heterogeneous clinical expressivity. Objective: To report clinical variants and sociodemographic characteristics in patients with MF under the care of a dermatological hospital. Methods: 290 patients with MF clinical and histopathological diagnosis attended to over the course of 11 years were included. Sociodemographic description of patients was made, who were classified according to clinical and histopathological variants. Results: MF was recorded in 57.9 % of women and 42 % of men. The most common clinical variant was the classic type in 46.2 %; dyschromic variants accounted for 35.2 %, out of which hypopigmented MF was the most representative (17.6 %); poikilodermatous MF accounted for 4.1 %, and folliculotropic, for 3.1%. The papular variant occurred in six patients (2.1 %), the single-plaque variety in three (1%), and the ichthyosiform, syringotropic and granulomatous slack skin varieties occurred in one patient each. The granulomatous variant was found in 0.7 %, and 1.4 % had erythroderma. Conclusions: The most common MF clinical variant was classic plaque stage, followed by dyschromic variants. Other clinical variants accounted for 18.6 %.

Síndrome de Sézary. Etiopatogenia, epidemiología, clínica, diagnóstico y pronóstico / Sézary syndrome Pathogenesis, epidemiology, clinical features, diagnosis and prognosis

El síndrome de Sézary (SS) es una rara y agresiva variante leucémica del linfoma cutáneo de células T, de pronóstico ominoso. Se caracteriza por presentar la tríada eritrodermia, linfadenopatías y linfocitos T neoplásicos circulantes. El diagnóstico está dado por la clínica, el estudio histopatológico, la citometría de flujo y el reordenamiento genético del receptor del linfocito T. En esta revisión se analizan la presentación clínica, la histopatología, el diagnóstico y el pronóstico de este síndrome. (AU)

Sézary syndrome (SS) is a rare and aggressive leukemic cutaneous T-cell lymphoma with poor prognosis. Is characterized by a triad of erythroderma, lymphadenopathy and circulating neoplastic T cells. Diagnosis is made by clinical features, histopathology, flow cytometry and T-cell receptor gene rearrangements. In this review we will analyze clinical presentation, histopathology, diagnosis and prognosis of SS. (AU)

Eritrodermia y Linfomas Cutáneo de Células T / Erythroderma and Cutanous T-Cell Lymphomas / Linfomas de células T e erâmulas

RESUMEN La eritrodermia es una erupción eritematosa más o menos escamosa que afecte a más del 90% de la superficie corporal. Se trata de una enfermedad capaz de comprometer el pronóstico vital y que puede complicarse con desequilibrios hidroelectrolíticos, trastornos de la termorregulación, infecciones, así como con fallo cardiovascular. En la presente publicación referimos el caso de un paciente masculino de 72 años con antecedentes de hipertensión arterial y de Lepra que recibió poliquimioterapia, es ingresado en el servicio de dermatología en el período de observación, pues hace alrededor de un año comenzó a presentar enrojecimiento de la piel y múltiples escamas. Al momento del examen se observa paciente eritrodérmico, ectropión, distrofia ungueal importante, con adenopatías palpables axilares e inguinales, asociado un prurito intenso generalizado. Se realizan estudios para el diagnostico incluyendo biopsia de ganglios inguinal y de piel, esta última compatible con Micosis Fungoide. Actualmente se reconocen tres variantes eritrodérmicas en el linfoma cutáneo de células T: el síndrome de Sézary, la Eritrodermia en micosis fungoide y la Eritrodermia en linfomas cutáneos de células T:No Especificada. Si bien se entiende al síndrome de Sézary como una expresión leucémica del linfoma cutáneo de células T eritrodérmico, con numerosas células de Sézary en piel, sangre y otros tejidos, la Eritrodermia en micosis fungoide se determinaría ante la ausencia de estos mismos hallazgos hematológicos e histológico ante un cuadro clínico eritrodérmico. Se hace necesaria la publicación del artículo por la forma de presentación de la micosis fungoide, teniendo en cuenta que esta dermatosis tiene un sin número de diagnósticos diferenciales que la hace la gran simuladora en la Dermatología.

ABSTRACT Erythroderma is a more or less scaly erythematous rash that affects more than 90% of the body surface area. It is a disease capable of compromising the vital prognosis and that can be complicated by hydroelectrolytic imbalances, thermoregulation disorders, infections, as well as cardiovascular failure. In this publication we refer to the case of a 72-year-old male patient with a history of high blood pressure and leprosy who received polychemotherapy, is admitted to the dermatology service in the observation period, as about a year ago he began to develop redness of the skin and multiple scales. At the time of the examination, an erythrothermal patient, ectropion, important ungueal dystrophy, with axillary and inguinal palpable adenopathies, associated with a widespread intense itching, is observed. Studies are conducted for diagnosis including inguinal and skin node biopsy, the latter compatible with Mycosis Fungoide. Three erythrodermal variants are currently recognized in skin T-cell lymphoma: Sézary syndrome, Erythroderma in fungoid mycosis, and Erythroderma in skin T-cell lymphomas: Undepede. While Sézary syndrome is understood as a leukemia expression of erythrodermal T-cell skin lymphoma, with numerous Sézary cells in skin, blood and other tissues, Erythroderma in fungoid mycosis would be determined in the absence of these same haematological and histological findings before an erythrodermal clinical picture. It is necessary to publish the article by the form of presentation of fungoid mycosis, taking into account that this dermatosis has a number of differential diagnoses that makes it the great simulator in Dermatology.

RESUMO Eritrodermia é uma erupção cutânea mais ou menos erethema que afeta mais de 90% da superfície do corpo. É uma doença capaz de comprometer o prognóstico vital e que pode ser complicada por desequilíbrios hidroelegóticos, distúrbios termoreguladores, infecções, bem como insuficiência cardiovascular. Nesta publicação, refere-se ao caso de um paciente do sexo masculino de 72 anos com histórico de pressão alta e hanseníase que recebeu poliquimoterapia, é internado no serviço de dermatologia no período de observação, pois há cerca de um ano começou a desenvolver vermelhidão da pele e múltiplas escamas. No momento do exame, observa-se um paciente eritrótermal, ectropion, importante distrofia ungueal, com adenopatias palpáveis axilares e inguinais, associadas a uma coceira intensa generalizada. São realizados estudos para diagnóstico, incluindo biópsia inguinal e nódulo da pele, este último compatível com Mycosis Fungoide. Três variantes eritromicais são atualmente reconhecidas em linfoma de células T da pele: síndrome de Sézary, Erythroderma na micose fungoide, e Erythroderma em linfomas de células T da pele: Undepede. Enquanto a síndrome de Sézary é entendida como uma expressão de leucemia de linfoma de pele de células T eritorodérmica, com numerosas células Sézary na pele, sangue e outros tecidos, eritrorgema em micose fungoide seria determinada na ausência desses mesmos achados haematológicos e histológicos diante de um quadro clínico eritroxermal. É necessário publicar o artigo sob a forma de apresentação da micose fungoide, tendo em vista que essa dermatose possui uma série de diagnósticos diferenciais que o torna o grande simulador em Dermatologia.

Eighty-two year old female with long term abdominal pain, fever and skin lesions. / Mujer de 82 años con dolor abdominal, fiebre y lesiones cutáneas de larga evolución.

We report the case of an old woman, consulting for fever, abdominal pain and constitutional symptoms one year of evolution. The differential diagnosis is between infectious, tumoral, or inflammatory disease, which may be located at the abdominal level, performing additional tests to rule out abdominal process. The existence of pain in the legs and level scan left thigh of a mass of hard consistency, makes us raise another diagnosis. Finally show on ultrasound soft tissue inflammatory changes regarding panniculitis. From this finding aetiologies of panniculitis are reviewed. Skin biopsy that shows the final diagnosis is made.