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.

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.

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.

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.

Linfoma cutâneo primário difuso de grandes células B, tipo perna ­ rápida evolução e desfecho desfavorável: relato de caso / Diffused primary cutaneous lymphoma of large B cells, leg type - quick evolution and unfavorable outcome: case report

Linfomas cutâneos primários são a segunda forma mais comum de linfomas extranodais, sendo os linfomas de células B, (CBCLs) representantes de 20 a 30% dos casos. O linfoma cutâneo difuso de grandes células B, Tipo Perna (PCDLBCL-LT), representa o tipo mais agressivo de CBCLs. Na maioria dos casos, a apresentação clínica é caracterizada por placas ou tumores solitários, ora ulcerados, em uma ou ambas as pernas, de rápido crescimento. O diagnóstico é confirmado através do estudo histopatológico e imunohistoquímico. O tratamento é realizado por meio de quimioterapia e seu prognóstico é reservado com uma sobrevida de 50% a 60% em 05 anos. O objetivo deste trabalho é relatar um caso atendido de linfoma cutâneo primário difuso de grandes células B, tipo perna em um paciente de 75 anos, do sexo masculino com apresentação clínica clássica e desfecho desfavorável, realizar uma revisão bibliográfica do período de 2010 a 2020 na base de dados PUBMED sobre o assunto, dada sua raridade e agressividade ímpar. As informações foram obtidas através de revisão do prontuário, registro fotográfico e revisão da literatura. Por tudo isso, pode-se concluir a importância de estudos multidisciplinares, envolvendo dermatologistas, hematologistas, oncologistas e patologistas para que o diagnóstico e tratamento sejam instituídos o mais precoce possível, visto a raridade e agressividade do PCDLBCL-LT. [au]

Primary cutaneous lymphomas are the second most common form of extranodal lymphomas; with B cell lymphomas (CBCLs) representing 20 to 30% of cases. Diffuse cutaneous large B cell lymphoma, leg type (PCDLBCL-LT), represents the most aggressive type of CBCLs. In most cases, the clinical presentation is characterized by solitary plaques or tumors, sometimes ulcerated, on one or both legs, of rapid growth. The diagnosis is confirmed through histopathological and immunohistochemistry studies. Treatment is carried out through chemotherapy and its prognosis is reserved with a 50% to 60% survival in 5 years. The objective of this work is to report a case of diffuse primary B-cell cutaneous lymphoma, leg type in a 75-year-old male patient with a classic clinical presentation and unfavorable outcome, to perform a literary review from 2010 to 2020 in the PUBMED database on the subject, given its rarity and unique aggressiveness. The data was obtained by reviewing the medical record, photographic record and literature review. For all this, it is possible to conclude the importance of multidisciplinary studies, involving dermatologists, hematologists, oncologists and pathologists so that the diagnosis and treatment are instituted as early as possible, given the rarity and aggressiveness of the PCDLBCL-LT. [au]

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 %.

[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.

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 %.

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.

Micosis fungoide y VIH ¡Es posible esta excepcional relación! / Mycosis fungoides and HIV.This exceptional relationship is possible! Case report

RESUMEN Las enfermedades cutáneas en pacientes con el virus de la inmunodeficiencia humana (VIH) y/o síndrome de inmunodeficiencia adquirida (SIDA) son comunes y altamente incapacitantes; sin embargo, la micosis fungoide (MF) es una complicación inusual en pacientes con VIH, por tal motivo no ha sido completamente dilucidado su etiopatogénesis ni su forma de presentación o manifestaciones clínicas en pacientes con esta patología. Se presenta el caso de una paciente con diagnóstico de VIH y MF atendida en la unidad de Dermatología del Hospital Pablo Tobón Uribe. Medellín - Colombia.

ABSTRACT Cutaneous diseases in patients with the human immunodeficiency virus (HIV) are common, becoming highly disabling entities in patients with acquired immunodeficiency syndrome (AIDS); however, mycosis fungoides (MF) is a complication of low occurrence in patients with HIV, so it has not been clearly elucidated its presentation or manifestations in patients with this pathology. In this report we present the case of a patient with a diagnosis of HIV and MF treated at the Dermatology Unit of the Hospital Pablo Tobón Uribe, in Medellín, Colombia.

Pitiriasis rubra pilaris tratada con lámpara excímero / Pityriasis rubra pilaris treated with excimer lamp

Resumen La pitiriasis rubra pilaris, es una dermatosis inflamatoria papuloescamosa e hiperqueratósica de origen desconocido y de progresión crónica, la cual puede evolucionar incluso a eritrodermia. El presente caso trata de un paciente de 27 años portador del virus de inmunodeficiencia humana, diagnosticado con pitiriasis rubra pilaris tipo IV, inicialmente tratado con corticosteroide tópico y fototerapia, por cuatro meses. Sin embargo, presentó reactivación de las lesiones, por lo que se recurrió a la aplicación de lámpara excímero, utilizada en otras patologías dermatológicas, mas no de uso habitual en la pitiriasis rubra pilaris.

Abstract Pityriasis Rubra Pilaris is an inflammatory papulosquamous and hyperkeratic dermatosis of unknown cause and chronic progression which can envolve even into erythroderma. This case deals with a 27-year old male patient carrier of VIH who was diagnosed with PRP type IV. Initially, it was treated with topical corticosteroid and phototherapy for four months. However, it showed reactivation of the injuries; therefore, excimer lamp was employed, which is used in other dermatologic pathologies but it is not a regular treatment for PRP type IV.

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.

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)

Primary multifocal anaplastic large cell lymphoma with eyelid involvement: a case report / Linfoma anaplásico de grandes células primário multifocal com envolvimento de pálpebra: relato de caso

ABSTRACT Ocular adnexal involvement in CD30+ lymphoproliferative disorders is rare. We report the case of a 73-year-old woman with a relapsing primary cutaneous anaplastic large cell lymphoma on her eyelid. A systemic extension study excluded extracutaneous involvement. Systemic chemotherapy resulted in an optimal response, with complete regression of the cutaneous lesions. There has been no recurrence during the 2 years of follow-up.

RESUMO O acometimento ocular adicional nos distúrbios linfoproliferativos CD30+ é raro. Relatamos o caso de uma mulher de 73 anos com linfoma de grandes células anaplásicas primárias recidivantes em sua pálpebra. A avaliação sistêmica excluiu envolvimento extracutâneo. A quimioterapia sistémica resultou em uma resposta ótima, com regressão completa das lesões cutáneas. Não houve recidiva durante 2 anos de acompanhamento.

Reticulose pagetoide em um cão: relato de caso / Pagetoid reticulosis in a dog: case report

Reticulose pagetoide é a expressão utilizada para uma das mais raras formas de linfoma descritas em cães. Trata-se de um distúrbio linfoproliferativo cutâneo que emerge de linfócitos T intraepidérmicos. Descreve-se aqui um caso de reticulose pagetoide com apresentação localizada, muito semelhante à doença de Woringer-Kolopp dos humanos, que acometeu um cão, Boxer, fêmea de 10 anos de idade. O paciente foi atendido devido ao aparecimento de um nódulo na região nasolabial, com evolução clínica de cerca de 30 dias. Histologicamente o nódulo era composto por uma proliferação linfoide intraepidérmica e anexal. Os linfócitos neoplásicos, células T confirmadas por meio da imunomarcação positiva para CD3 e negativa para CD79, eram grandes células redondas e monomórficas. Apesar de rara, a reticulose pagetoide é um distúrbio linfoproliferativo de fácil suspeita com base apenas na morfologia celular. Esse neoplasma deverá ser lembrado sempre que um padrão linfoide intraepidérmico estiver presente.(AU)

Reticulosis pagetoide is an expression used for one of the rare forms of lymphoma described in dogs. It is a cutaneous lymphoproliferative disorder that emerges from intraepidermal T-cells. We describe a case of pagetoid reticulosis with localized presentation, very similar to Woringer-Kolopp's disease in humans, which affected a 10-year-old Boxer female. The patient was seen due to the appearance of a nodule in the nasolabial region with clinical evolution of about 30 days. Histologically, it was composed of an intraepidermal and annexal lymphoid proliferation. Neoplastic lymphocytes, T-cells confirmed by CD3-positive and CD79-negative immunolabeling, were large, round, and monomorphic cells. Although rare, pagetoid reticulosis is an easily suspected lymphoproliferative disorder based only on cell morphology. This neoplasm should be remembered whenever an intraepidermal lymphoid pattern is present.(AU)

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.

Linfoma anaplásico de células T grandes primario cutáneo CD30+. Serie de nueve casos / Primary cutaneous CD30+ anaplastic large T-cell lymphoma. Series of nine cases

Resumen 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.

Abstract 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.

Micosis fungoide en vulva: Presentación de un caso / Vulvar mycosis fungoides: A case report

Mycosis fungoides is a malignant neoplasm originating in T lymphocytes. It usually starts with the appearance of flat spots, called "patch stage". As time goes by, lesions infiltrate surrounding tissue and plaques appear ("plaque stage"). We present the case of a 50-year-old female patient wiht clinical and histological findings of vulvar mycosis fungoides in patch stage.

La micosis fungoide es una neoplasia maligna originada en los linfocitos T. Usualmente inicia con la aparición de manchas planas o estadio de mancha. Con el correr de los años, las lesiones van siendo infiltrantes, apareciendo las placas o estadio de placas. Presentamos a un paciente de sexo femenino de 50 años con hallazgos clínicos e histopatológicos de micosis fungoide en estadio eritematoso o manchas a nivel de vulva.