Steatocystoma Multiplex in a Family

ABSTRACT

Steatocystoma multiplex is a rare, inherited disorder that is characterized by multiple, asymptomatic, variably sized dermal cysts. The condition is transmitted in an autosomal dominant fashion; although sporadic cases have been documented. Keratin 17 has been proposed to be an important factor in inherited steatocystoma. In this study, a 29-year old man has a 4-year history of asymptomatic, movable, skin-colored nodules on his face, neck, scalp, anterior chest and back. His father and elder-brother have similar lesions. Histologically, the cysts show a thin stratified squamous epithelium with sebaceous glands arising from its wall and an absence of the granular cell layer. Generally, there are two treatments-medical treatment and surgical treatment. In case of non- inflamed lesions, surgical excision or drainage is regarded as the best treatment. We tried excisional biopsy and until now there has been no recurrence in the operation area over the past 12 months following the operation.