Multiple cylindromas may cover almost the entire scalp, causing the disfiguring turban tumor appearance and necessitating extensive plastic surgery. No racial disparity is reported. Multiple, inherited cylindromas usually begin in early adulthood and may progress through time. The solitary form usually begins in middle age or later as a slow-growing, rubbery nodule exhibiting no symptoms. The dominantly inherited, multiple variety appears shortly after puberty as numerous, rounded nodules of various sizes.
Additional lesions develop over time.
Except for the Brooke-Spiegler syndrome, pertinent findings are limited to the skin. Solitary lesions are firm, rubbery nodules with pink, red, or sometimes blue coloring ranging in size from a few mm to several cm. The solitary form is nearly always found on the scalp, head, and neck. The multiple form most frequently occurs on the scalp, head, and neck and can develop on the trunk and extremities. The multiple form is autosomal dominantly inherited and is implicated on the cyld 1 gene on chromosome 16.