A Case of Malignant Histiocytosis Showing Papulosquamous Skin Lesions and Fever as Initial Manifestations

ABSTRACT

We report a case of malignant histiocytosis which began with intermittent fever and scaly skin lesions. A 3-year-old girl presented with erythematous scaly papules on the face and the trunk, and high fever for 3 months. The cutaneous lesions consisted of widespread coin-sized erythematous scaly papules with marginal brownish pigmentation. She was anemic and thrombocytopenic and had impairment of the liver function. Histopathologic study of the skin lesions showed non-specfic findings except for hyperkeratosis. However, bone marrow examination revealed an increased number of histiocytes, mostly immature with active phagocytosis of erythroid cells, myeloid cells, and platelets. She was diagnosed as having malignant histiocytosis and treated with cyclophosphamide and vincristine. She died the next day after the treatment had begun.