An 8-year-old girl presented with complaints of inability to stand from sitting position for 3 months and inability to lift her head for one month. Her past, history was insignificant. Examination revealed periorbital puffiness, hypertrichosis, papular lesions over skin of hands, wrist and elbow bilaterally. There was genralized hypotonia with a positive Gower’s sign. She had burning sensation and redness on tips of fingers. The dorsum of hands had Gottron’s papules (Fig. 1) and scaling eczematoid dermatosis. Other systems were normal. Her CPK levels were 953IU, LDH was 4496 IU, SGOT was 450 IU and ESR was 75 mm/hr. ANA was negative. The muscle biopsy showed inflammatory changes with perivascular infiltration and occlusion of microvasculture with perifascicular atrophy. A diagnosis of Juvenile Dermatomyositis was made. The patient was treated with prednsiolone and physiotherapy.

Fig.1. Atrophic and scaly papular lesions (Gottron papules) over knuckles.

Juvenile Dermatomyositis is a systemic vasculopathy with involvement of skin and focal area of myositis resulting in proximal muscle weakness with an incidence of 3-4 per million children per year. The etiology of the condition is unclear yet. There may be involvement of joints, kidney, brain, lung and intestine. Most recover in 6 months to 2 years. Recurrence is rare. Those who don't respond to steroids may be treated with azothioprine, methotrexate or cyclosporine as adjuvant agents.