Fatal vascular occlusion in juvenile dermatomyositis

Juvenile dermatomyositis (JDMS) is a relatively rare disease characterised by vasculopathy.1-4 Involvement of the gastrointestinal tract may occur in some subjects and is often life threatening. We describe here a case of fatal JDMS with gastrointestinal perforation. Immunohistochemical examination by antibody against factor VIII seems to be useful for evaluating the pathological basis of vasculopathy in JDMS. A 13 year old Japanese girl was admitted in April 1994 with high fever, muscle pain, and muscle weakness. She noticed a facial rash for two months before admission. Physical examinations were; blood pressure 135/90 mm Hg, temperature 38°C, and weight 50 kg. She presented with an erythematous rash on her face, neck and arms, heliotropic eruption, Gottron’s sign, and nail fold telangiectasia. Proximal muscular weakness and pain were prominent. Laboratory findings were as follows; stool occult blood negative, leucocyte count 5800/mm3, erythrocyte count 4800 × 103/mm3, thrombocyte count …