RESUMO
Resumen Se presenta una paciente femenina con erupción papulosa generalizada que compromete cara, tronco y cuatro miembros. En el examen físico se visualizaengrosamientoy oscurecimiento de la piel. Se realiza el estudio integral y el correspondiente diagnóstico diferencial.El estudio histopatológico cutáneo exhibió un incremento excesivo de mucina intersticial, actividad fibroblástica y engrosamiento de los haces de colágeno. Se arriba al diagnóstico de escleromixedema debido a las manifestaciones cutáneas características. Se constata compromiso extracutáneo en ausencia de gammapatía monoclonal. Se indica prednisona, talidomida ehidroxicloroquina con excelente evolución.
Abstract A female patient presents with a generalized papular rash involving face, trunk, and four limbs. The skin is thickened and darkened, forming yellowish erythematous plaques that are linearly arranged papules. It is assumed as a generalized sclerodermiform syndrome and a comprehensive study and corresponding differential diagnosis is performed. The histopathological study of the skin showed an excessive increase of interstitial mucin, fibroblast activity and thickening of collagen bundles. The characteristic clinical expression and the histopathological study added to the extra cutaneous involvement lead to the diagnosis of scleromyxedema. There was no evidence of monoclonal gammopathy. Prednisone, thalidomide and hydroxychloroquine are indicated with excellent evolution.
Assuntos
Humanos , Feminino , Adulto , Diagnóstico Diferencial , Escleromixedema/terapia , Manifestações Cutâneas , Escleromixedema/diagnósticoRESUMO
Abstract: Cutaneous mucinoses are a heterogeneous group of dermatoses in which excess deposition of mucin in the dermis gives the skin a waxy appearance, with papules and plaques that can vary from self-healing mucinosis to even disrupting the normal shape of a patient's face, conferring a leonine facies, or be part of life threatening diseases like scleromyxedema. This review will describe the most recent classification on lichen myxedematosus in the generalized (scleromyxedema) and the localized forms, as well as the different organ systems involved in scleromyxedema, diagnostic workup, current management, and prognosis.