RESUMO
BACKGROUND: Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, typically presents in middle-aged to elderly individuals. OBJECTIVE: We sought to study the demographics, clinicopathologic features, treatment response, and prognosis of patients with biopsy-proven MF diagnosed before 20 years of age. METHODS: Patients were identified from a prospectively collected database for retrospective analysis. RESULTS: Of 1902 patients with MF, 34 had juvenile-onset MF: 41% were stage IA, 56% were stage IB, and 3% were stage IIB at diagnosis. The male to female ratio was 1.1:1. The median age of symptom onset was 9 years (range 3-19 years), with a delay in diagnosis between 1 month and 14 years. Patients primarily presented with hypopigmented (53%), hyperpigmented (29%), and pink-violaceous (41%) patches/plaques. Immunohistochemistry revealed 39% with CD8(+) immunophenotype, 67% of which had hypopigmented lesions. The phototherapy response rate in 21 patients was 81%. All patients who completely responded to narrowband ultraviolet B phototherapy had hypopigmented MF. LIMITATIONS: This is a single cancer center study. CONCLUSION: Juvenile-onset MF presents with early-stage disease with an overrepresentation of hypopigmented MF and CD8(+) immunophenotype. Narrowband ultraviolet B is an effective treatment option for juveniles, especially for those with the hypopigmented variant.
Assuntos
Micose Fungoide , Terapia PUVA/métodos , Neoplasias Cutâneas , Adolescente , Idade de Início , Biópsia , Criança , Pré-Escolar , Estudos Transversais , Bases de Dados Factuais , Feminino , Humanos , Hiperpigmentação/imunologia , Hiperpigmentação/patologia , Hipopigmentação/imunologia , Hipopigmentação/patologia , Imunofenotipagem , Masculino , Micose Fungoide/tratamento farmacológico , Micose Fungoide/imunologia , Micose Fungoide/patologia , Prognóstico , Estudos Retrospectivos , Pele/patologia , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/patologia , Resultado do Tratamento , Terapia Ultravioleta/métodos , Deficiência de Vitamina D/imunologia , Deficiência de Vitamina D/patologia , Adulto JovemRESUMO
Three healthy children with an anogenital skin eruption, all of whom had undergone extensive medical evaluation and treatment, had undetected intestinal Crohn's disease. Biopsy of the skin showed noncaseating granulomas consistent with cutaneous Crohn's disease. Inflammatory bowel disease should be in the differential diagnosis of persistent nontender, erythematous papules, nodules, and plaques in the anogenital region.
Assuntos
Doenças do Ânus/diagnóstico , Doença de Crohn/diagnóstico , Doenças dos Genitais Femininos/diagnóstico , Doenças dos Genitais Masculinos/diagnóstico , Dermatopatias/diagnóstico , Doenças do Ânus/tratamento farmacológico , Doenças do Ânus/patologia , Criança , Pré-Escolar , Doença de Crohn/tratamento farmacológico , Doença de Crohn/patologia , Fármacos Dermatológicos/uso terapêutico , Diagnóstico Diferencial , Feminino , Doenças dos Genitais Femininos/tratamento farmacológico , Doenças dos Genitais Femininos/patologia , Doenças dos Genitais Masculinos/tratamento farmacológico , Doenças dos Genitais Masculinos/patologia , Granuloma/diagnóstico , Humanos , Masculino , Mesalamina/uso terapêutico , Metronidazol/uso terapêutico , Prednisolona/uso terapêutico , Dermatopatias/tratamento farmacológico , Dermatopatias/patologiaRESUMO
We present a case of a 14-year-old boy with a large ulcerated plaque on the scalp for 6 months, who was found to have primary cutaneous CD30-positive, anaplastic kinase-negative, anaplastic large cell lymphoma with post-auricular lymphadenopathy. MRI, bone marrow biopsy, and laboratory data demonstrated no other systemic involvement. He was treated with radiation and low-dose oral methotrexate, with improvement of the lesion and lymphadenopathy. Very few cases of primary cutaneous CD30-positive anaplastic large cell lymphoma in the pediatric population have been reported, and our case represents one of the first pediatric patients with local lymph node involvement.
