A Case of Crow-Fukase Syndrome Associated with Periodic Fever and Severe Thrombocytopenia

Abstract

A 51-year-old male was admitted because of muscle weakness and paresthesia of extremities. The eruptions with itching and pigmentation were recognized on his face and back. A cerebrospinal fluid examination revealed protein-cell dissociation, peripheral nerve verocity was delayed, and neurological investigations revealed polyneuropathy of glove and stocking type. A bone marrow aspiration showed slight proliferation of plasma cells. Decreased testslerone and increased FSH were pointed out by endocrinological analysis. A right subclavian lymphnode biopsy revealed a proliferation of plasma cells of germinal center, and a diagnosis of Crow-Fukase syndrome was made. We started a therapy with prednisolone and melphalan, but periodic fever and severe thrombocytopenia occurred. Infections and side effects of drugs were denied. So we suspected these symptoms had a relation to Crow-Fukase syndrome and VENP therapy was started. A temporary remission was obtained. But he died of respiratory distress after several recurrence of these symptoms.