Clinical manifestations of 102 cases with the Crow- Fukase syndrome (the syndrome of polyneuropathy, anasarca, skin changes, endocrinopathy, dysglobulinemia, and organomegaly), with or without myeloma, were reviewed. Fifty-six cases with myeloma consisted of 31 with osteosclerotic, 17 with mixed osteosclerotic and osteolytic, and 8 with osteolytic. Forty-six cases without myeloma consisted of 2 with extramedullary plasmacytoma, 33 with M protein alone, and 11 with polyclonal protein alone. There was no significant difference in incidence of the major clinical manifestations between the two groups with and without myeloma. They had a common characteristic histologic finding of the lymph node resembling that of Castleman's disease.
A syndrome of progressive muscle spasm, alopecia, and diarrhea was seen in 15 patients. The syndrome was characterized by painful intermittent muscle spasm, alopecia, amenorrhea, and malabsorption, and was sometimes associated with epiphyseal destruction and retarded growth. Symptoms began at age 10 and were more common in women than men. Muscle cramps affected the limbs first and then, several years after onset, the neck, trunk, and masticatory muscles. The course was progressive and led to malnutrition. Four patients died from 5 to 18 years after onset. Autopsy revealed polypoid changes throughout the gastrointestinal tract.
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