Combination of massive mucinosis, dermatomyositis, pyoderma gangrenosum-like ulcer, bullae and fatal intestinal vasculopathy in a young female.

Cutaneous mucinosis secondary to autoimmune collagen vascular disease is well recognized, but manifestation as cellulitis-like massive cutaneous mucinosis preceding dermatomyositis is unusual. Here we report a 21-year-old Taiwanese woman with a large, rapid onset, painful erythematous, edematous plaque, which histopathologically revealed septal panniculitis with fat necrosis and massive mucin deposition. Incapacitated muscle weakness of proximal extremities, generalized edema, heliotrope erythema, and Gottron's papules developed in a short period of time with high titers of serum muscle enzyme. Serological titers of ANA, anti-dsDNA, anti-ENA panels, and erythrocyte sedimentation rate, however, all showed unremarkable results. Diagnosis of dermatomyositis was confirmed by electromyographic findings of myopathy. As the disease progressed, large, deep cutaneous ulceration and vesiculobullous lesions also developed. In spite of aggressive treatment, the patient died 9 months after the disease onset, probably due to the complication of gastrointestinal ischemia and perforation.