RESUMO
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Assuntos
Humanos , Feminino , Gravidez , Adulto , Hanseníase Multibacilar/complicações , Hanseníase Multibacilar/diagnóstico , Hanseníase Multibacilar/patologia , Prurigo/diagnóstico , Prurigo/prevenção & controle , Gravidez/metabolismo , Gravidez/fisiologia , Doenças Endêmicas/prevenção & controle , Escleromixedema/complicações , Escleromixedema/diagnóstico , Escleromixedema/terapia , Urticária/diagnóstico , Urticária/prevenção & controle , Urticária/terapia , Biópsia/instrumentação , Biópsia/métodos , BiópsiaAssuntos
Alopecia/etiologia , Sobrancelhas , Escleromixedema/diagnóstico , Derme/patologia , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
Scleromyxedema (SM) is a rare chronic progressive and highly intractable cutaneous disease with unknown etiology, affecting both genders equally between 30 and 50 years. The disease is characterized with mucin deposits in the skin and/or other organs. In fact it is a clinicopathological subset of lichen myxedematosus (LM) according to a new classification. Sclerodermiform plaques and lichenoid papules are characteristic cutaneous lesions. An elevation of IgG λ (lambda) chain exists in most cases and extracutaneous involvement occurring with variable systemic findings is also detected. Generalized form is quite difficult to treat and may even be fatal. Herein, we present a male patient with typical features of generalized papular and sclerodermoid LM variety and with benign outcome by isotretinoin.
Assuntos
Erupções Liquenoides/patologia , Escleromixedema/patologia , Pele/patologia , Biópsia , Fácies , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
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.