Juvenile dermatomyositis with gingival vasculopathy

A 6-year-old boy with juvenile dermatomyositis (JDM) positive for anti-TIF1y antibodies for 3 years on methotrexate (1 mg/kg) reported with recurrent heliotrope and Gottron’s rashes, and muscle weakness refractory to corticosteroids (1 mg/kg) for 3 months. He progressively developed periorbital edema, oral ulcers, and skin ulcers over the trunk. On examination, Gottron’s papules, periungual desquamation, ulcerative rash on the anterior of the middle finger, ulcerative rash over the back and umbilicus, periorbital edema with partly healed heliotrope rash, and gingival vasculopathy (Fig. 1) were noted. The gingival changes may be surrogate for an underlying vasculitis which signifies severe JDM, also supported by the poor response to high-dose corticosteroids. Therefore, the child was treated with intravenous methylprednisolone and rituximab. Over the next 5 months, the child gradually improved with complete resolution of the heliotrope rash, gingival vasculopathy, skin ulcers, and periungual rash.