Definition

A rare, inherited disorder in which tumors form in the hair follicles and sweat glands in the skin. The tumors usually occur on the head and neck, but they can also occur in the salivary glands and in other parts of the body. They tend to be large and increase in number over time and can affect vision, hearing, sense of smell, and the ability to chew and swallow. The tumors usually appear in late childhood or early adolescence. They are usually benign (not cancer) but may become cancer. People with this disorder have an increased risk of developing basal cell skin cancer and salivary gland tumors. Brooke-Spiegler syndrome is caused by a mutation (change) in the CYLD gene. Also called BSS.