Undermining Ulcers in Juvenile Dermatomyositis.

Abstract

A 6-year-old boy with a history of refractory anti–transcription intermediary factor 1-γ (anti–TIF1-γ) juvenile dermatomyositis presented to the rheumatology clinic with skin ulcers of 10 days in duration. Physical examination revealed normal muscle for strength. Heliotrope rash, Gottron sign, and diffuse erythematous eruption on the neck, trunk, and buttocks were observed (Figs. 1A, B). No calcinosis cutis, and 2 ulcers (4 × 3 cm, 1 × 1 cm) on the left upper arm deep into the underlying biceps with well-circumscribed margins were observed (Fig. 2). Laboratory studies including muscle enzymes were unremarkable. Patients with anti–TIF1-γ juvenile dermatomyositis tend to have chronic or treatment-resistant disease course. Undermining ulcer is a severe cutaneous manifestation associated with vasculitis or vascular damage due to panniculitis or calcinosis. After 2 cycles of monthly intravenous immunoglobulin therapy (~1.7 g/kg/month) along with continuing use of prednisone and thalidomide at previous dose, the ulcers healed completely (Fig. 3), and other skin lesions improved significantly as well.FIGURE 1: Gottron sign (A) and diffuse erythematous eruption on the buttocks (B).FIGURE 2: Undermining ulcers on the left upper arm.FIGURE 3: The ulcers healed completely after 2 months of treatment.Differential diagnosis: Pyoderma gangrenosum Pressure ulcer Buruli ulcer Dermatomyositis (anti–TIF1-γ, anti-MDA5)