Hereditary trichilemmal cysts

One hundred and fifteen individuals from 60 families are described who had trichilemmal cysts. These were solitary or multiple intradermal cysts and occurred most frequently on the scalp. A punctum leading from the skin surface to the cyst was not seen in any patient. Because they are encapsulated they are easily removed. Histologicaily they resemble the external root sheath in the region of the follicular isthmus and are quite different from epidermoid cysts. They are not associated with Gardner's syndrome or any other diseases. In 46 of the 60 families an autosomal dominant mode of inheritance was demonstrated.

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