Malignant histiocytosis (MH) is a rapidly progressive and usually fatal disorder characterized clinically fever, generalized weakness, lymphadenopathy, hepatosplenomegaly accompanied by jaundice, and purpura. The frequency of skin manifestations
has
been reportrd about 15% of MH patients. Papules, plaques and nodules are commonly met. Histopathologcally MH shows infiltrations of atypical histiocytes with various 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 alos skin with vaorious features ; erythematous papules, annular purpuric patches, subcutaneous nodules and hemorhagic nodules.
Immunohistochemically the majority of patients' atypical histiocytes in lesional skin shows α-1 antitrysin positive, but lysozyme negative. It may suggest that these are poorly differentiated atypical histiocytes rather than well differentiated
ones.
(Kor J Dermatol 1992;30(5) : 720-726)
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