RESUMEN
Scleromyxedema is a rare, generalized form of lichen myxedematosus, which may be associated with systemic involvement and can be fatal. The therapeutic options available provide partial or inconsistent response and are associated with significant adverse effects. We report a case of scleromyxedema with cardiac involvement, treated with low-dose intravenous immunoglobulin, with almost complete clearing of the skin lesions. The patient died after three cycles of treatment, possibly due to myocardial infarction.
Asunto(s)
Muerte Súbita Cardíaca/etiología , Cardiopatías/etiología , Inmunoglobulinas Intravenosas/efectos adversos , Factores Inmunológicos/efectos adversos , Escleromixedema/terapia , Adulto , Electrocardiografía , Resultado Fatal , Humanos , Inmunoglobulinas Intravenosas/administración & dosificación , Factores Inmunológicos/administración & dosificación , Masculino , Factores de Riesgo , Escleromixedema/diagnósticoRESUMEN
A 45-year-old male presented with asymptomatic tumors all over the body. The tumors showed no signs of ulceration or regression. There were generalized, nontender, firm to hard enlarged lymph nodes without hepatosplenomegaly. Biopsy and immunophenotyping revealed CD 30+ anaplastic primary cutaneous large cell lymphoma. Primary cutaneous anaplastic large cell lymphoma is characterized by single or grouped reddish-brown tumor nodules, which frequently tend to ulcerate. Secondary involvement of lymph nodes is seen in only 25%. The lesions responded dramatically to chemotherapy, but recurred.
Asunto(s)
Antígeno Ki-1/biosíntesis , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma de Células B Grandes Difuso/inmunología , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/inmunología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ciclofosfamida/uso terapéutico , Doxorrubicina/uso terapéutico , Humanos , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Prednisona/uso terapéutico , Neoplasias Cutáneas/tratamiento farmacológico , Vincristina/uso terapéuticoRESUMEN
Tuberous sclerosis is an autosomal dominant disease due to mutations in two genetic loci, characterized by hamartoma formation in the skin, nervous system, heart, kidney and other organs. Dyschromatosis universalis hereditaria is an autosomal dominant genodermatosis, characterized by small hyperpigmented and hypopigmented macules, uniformly distributed over the entire body. The face is rarely involved and the palms, soles and mucous membranes are usually spared. We report a case of tuberous sclerosis with dyschromatosis universalis hereditaria, with hyperpigmented and hypopigmented macules affecting the palms, soles and oral mucosa. To our knowledge, this is the first reported case of such an association.