Masouyé I, Chavaz P, Salomon D, Balderer J, Carraux P, Saurat J H
Clinique de Dermatologie, Hôpital Cantonal Universitaire, Geneva, Switzerland.
Pediatr Dermatol. 1990 Dec;7(4):299-302. doi: 10.1111/j.1525-1470.1990.tb01030.x.
A 2-month-old girl acutely developed a brownish, firm nodule on the left thigh. The lesion was surgically removed when the patient was age 3 months, and histology showed a massive dermal infiltration by large histiocytic cells with abundant ground glass cytoplasm. Most cells were S100 protein positive. Electron microscopic examination revealed the presence of Birbeck granules in about 30% of the histiocytes, as well as laminated dense bodies and wormlike bodies. No recurrence was observed during follow-up for three years. We believe that this case, like the one previously reported, represents a clinical variant of Hashimoto-Pritzker disease.