Steatocystoma multiplex is a rare disorder characterized by multiple, asymptomatic, yellow-coloured to skin-coloured cystic lesions localised mainly to the arms, chest, axillae and neck. It is an autosomal dominant inherited disorder, but most cases are sporadic. Mutations in KRT17 have been demonstrated in families presenting as steatocystoma multiplex. KRT17 encodes the type I intermediate filament chain keratin 17, expressed in nail bed, hair follicle, sebaceous glands, and other epidermal appendages.
