RESUMO
BACKGROUND: Scleromyxedema is a rare, para-neoplastic, chronic, progressive condition of the Lichen myxedematosus (LM) family. The clinical picture consists of generalized confluent papular eruptions with possible systemic manifestations, which may be fatal as it still constitutes a therapeutic dilemma. Histologically, it is characterized by dermal mucin deposition, fibroblast proliferation with fibrosis, with monoclonal gammopathy in the absence of thyroid disease. Some atypical forms of the disease were reported in the literature, but none were reported in acute leukemia. CASE PRESENTATION: Herein, we report a case of a 21 years old female patient, known case of acute lymphoblastic leukemia (ALL), who developed numerous hyper-pigmented erythematous papules and plaques, mainly over her thighs, lower abdomen, and sub-mammary flexures. Histopathology of skin lesions confirmed the diagnosis of atypical scleromyxedema. Her symptoms significantly improved with the use of high dose intravenous immunoglobulin (IVIG). CONCLUSIONS: Despite that scleromyxedema is associated with many hematologic disorders, it is very rarely associated with acute lymphoblastic leukemia, and a high index of suspicion is needed for diagnosis. IVIG remains a reasonable management of such a disabling disease.
Assuntos
Imunoglobulinas Intravenosas/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Escleromixedema/diagnóstico , Biópsia , Relação Dose-Resposta a Droga , Feminino , Humanos , Leucemia-Linfoma Linfoblástico de Células Precursoras/imunologia , Escleromixedema/tratamento farmacológico , Escleromixedema/imunologia , Escleromixedema/patologia , Pele/imunologia , Pele/patologia , Coxa da Perna , Resultado do Tratamento , Adulto JovemRESUMO
Vitamin B12 (cobalamin) deficiency is common in developing countries. Its dermatologic manifestations include hair and nail changes and glossitis. Cases of generalized hyperpigmentation associated with vitamin B12 deficiency have rarely been reported. Localized hyperpigmentation is less frequently described, affecting palms, soles, and flexural areas. We report a rare case of reversible melasma-like cutaneous hyperpigmentation associated with pernicious anemia and discuss the possible mechanisms of this association.