A 56-year-old female was admitted on May 2, 1979 for a persistent high fever, eczema and left exophthalmus of one month duration. On physical examination, left exophthalmus with chemosis and erythematous nodules of about 5 cm in diameter scattered through the trunk were noted, but neither superficial lymphadenopathy nor hepatosplenomegaly was seen. Blood counting showed mild pancytopenia with a few histiocyte-like cells with erythrophagocytosis. These atypical cells, also were found in a small number on the bone marrow smears. Skin biopsy revealed proliferation of atypical cells within capillaries and blood vessels of the skin. Morphological study showed that such atypical cells in the blood, bone marrow and the skin belonged to histiocytes, some of which were immature in dominancy of the nucleus with prominent nucleoli, but many of them were mature in erythrophagocytosis in their huge cytoplasm.
From the above-mentioned clinical manifestations and laboratory data, a diagnosis of malignant histiocytosis (Rappaport) was made. The patient was treated with combination therapy of vincristine and corticosteroids with a very transient effect, deteriorated rapidly, and died on June 11, 1979, two months after onset. Permission of autopsy was not obtained.
