1993 Volume 16 Issue 5 Pages 388-396
We report a 68 year-old man with overlap syndrome of progressive systemic sclerosis (PSS) and polymyositis (PM), who died of fetal hyperinfection with strongyloidiasis. In 1978, he developed muscle weakness and Raynaud's phenomenon. In 1982, he developed fever of 37_??_38°C and erythema with desquamation. He was diagnosed as erythroderma in the department of dermatology of our hospital in March 1987. He started receiving prednisolone at a daily dose of 5mg. However, in December 1987, he admitted to our hospital complaining of general fatigue. On admission, he revealed marked systemic scleroderma, pigmentation of the skin, muscle atrophy and weakness. Eosinophils were markedly increased in the peripheral blood. Serum IgE and CPK were also markedly elevated. Anti-nuclear antibodies were strongly positive. He was diagnosed as overlap syndrome with PSS and PM by biopsy of the skin and the muscle. Subsequently, treatment with 60mg/day of prednisolone was initiated. However, he developed abdominal pain, diarrhea, cough, hemosputum, followed by rapid progression of anemia and unconsciousness, and died in March 1988. Autopsy revealed the massive and diffuse bleeding in the bilateral lung, and the ulceration in the whole intestine with massive bleeding. Numerous filaria-form larva of strongyloides stercoralis were observed in the lung, intestine, liver, pancreas and mesenteric lymphnodes. In the literature, several cases of fetal hyperinfection with strongyloides stercoralis has been reported in immunocompromised host. However, there has been few reports concerning the hyperinfection of strongyloides stercoralis in patients with PSS or PM. Because of its poor prognosis, we should pay attention to the presence of strongyloidiasis in patients with collagen diseases when unexplained eosinophilia and elevated serum IgE were observed.
