Guess what! Multiple pilomatricomas and Steiner disease

An 18-year-old male adolescent was referred to our department for the presence of multiple skin nodules since the age of 9 years. At the age of 22 months the patient was diagnosed as having myotonic dystrophy (Steinert's disease). So far, nine lesions like these have been excised and histologically analysed, two located on the left arm, one on the neck and one on the scalp. A skin examination revealed three nodular lesions, two localised on the scalp (Fig. 1) and one on the trunk (Fig. 2); the lesions ranged from 0.5 to 3.5 cm, and were hard on palpation and asymptomatic, with elevated, smooth and teleangiectatic borders and a central invagination filled with whitish, firm, granular material. The lesions were all excised. The histological examination of all the biopsy specimens revealed basophilic cells at the periphery, and islets of shadow cells with an unstained central area at the site of lost nucleus (Fig. 3). The shadow cells were surrounded by a granulomatous infiltration composed mainly of giant cells.