A case of malignant histiocytosis with various skin manifestations / 대한피부과학회지

ABSTRACT

Malignant histiocytosis(MH) is a rapidly progressive and usally fatal disorder characterized clinically fever, generalized weakness, lymphadeno athy, hepatosplenomegaly accompanied by jaundice, and purpura. The frequency of skin manifestations has been repoterd about 15% of MH patients. Papules, plaques and niidules are commonly met. Histopathologically MH shows infiltrations of atypical histiocyte; with uarious maturity in many organs including skin. We present a case of a 32-year-old female with MH involving not only lymph node, liver, spleen and bone marrow but also skin with vaorious features ; erythematous papules, annular purpuric patches, subcutaneous nodules and hemomagic nodules. Immunohist,ochemically the majority of patients atypical histiocytes in lesional skin shows a-1 antitrysin positive, but lysozyme negative. It may suggest that these are poorly differentiated atypical histiocytes rather than well differentiated ones.