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Abstract
In a sibship of nine, six members had hereditary medullary carcinoma of the thyroid.
Two of those with thyroid neoplasms and two without had numerous small papular skin
lesions. These proved to be a type of pilar tumor that we 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 years. Fifteen of 37 members
older than the age of 25 years exhibited the typical skin lesions. Obviously, the
original sibship was the repository of two 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
firbrous tissue. Associated skin lesions in this kindred were trichodiscomas and acrochordons.