Localized cluster of nodules in childhood

An 8-year-old girl presented with multiple, asymptomatic skin lesions localized to her right lower abdomen. These had first appeared 6 years previously, and had gradually increased in number over the intervening years. The patient was otherwise systemically well. On physical examination, a plaque 50 mm in size was found on the patient’s right abdomen, made up of coalescing, firm, red-brown papules and nodules ranging from 4 to 10 mm in size (Fig. 1). Laboratory investigations gave normal results for full blood count, urea and electrolytes, liver function tests and protein electrophoresis. However, autoantibody screen revealed a positive titre of anti-smooth muscle antibodies (anti-SMA) (≥ 1 : 320); the rest of the autoantibody screen was negative.