Hereditary multiple fibrofolliculomas with trichodiscomas and acrochordons

Abstract

In a sibship of 9, 6 members had hereditary medullary carcinoma of the thyroid. Two of those with thyroid neoplasms and 2 without had numerous small papular skin lesions. These proved to be a type of pilar tumor that was named fibrofolliculoma. Further investigation of the total kindred of 70 showed no other evidence of thyroid neoplasm. Skin tumors only appeared after the age of 25 yr. Fifteen of 37 members older than the age of 25 yr exhibited the typical skin lesions. Obviously, the original sibship was the repository of 2 dominantly inherited traits. The fibrofolliculoma is characterized by abnormal hair follicles with epithelial strands extending out from the infundibulum of the hair follicle into a hyperplastic mantle of specialized fibrous tissue. Associated skin lesions in this kindred were trichodiscomas and acrochordons.