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July 4, 1977

Trichilemmomas in Cowden's Disease

Author Affiliations

New York Medical College Metropolitan Hospital Center New York
Wayne State University Detroit
Ohio State University Columbus

JAMA. 1977;238(1):26. doi:10.1001/jama.1977.03280010026004

To the Editor.—  Papules of the face and oral mucous membranes are the most characteristic lesions in Cowden's disease. Other changes include goiter, thyroid adenoma and carcinoma, hypothyroidism, hypertension, leiomyomas of the uterus, punctate keratoses of the palms, and vitiligo. Affected women have almost invariably had fibrocystic disease, carcinoma of the breast, or both. At least one previously reported patient who had fibrocystic disease1 is now known to have died of metastatic carcinoma of the breast. Histopathologic examination of the cutaneous growths has been said to show only nonspecific changes.1-4We have studied numerous biopsy specimens from several patients with Cowden's disease and have identified the cutaneous facial tumors as trichilemmomas, a benign neoplasm of the lower outer root sheath consisting of poorly keratinized, glycogen-rich cells surrounded by a vitreous membrane.5-7 In a report of 40 trichilemmomas,5 no instance of multiple tumors was uncovered except in