Abstract Three consecutive generations of a family have had members affected with trichoepitheliomas and milia. One member, the proband, also had cylindromas. The pattern of inheritance was a dominant one, with only the male members affected. Histopathologically, the trichoepitheliomas and milia contained keratinizing cysts with laminated centers, peripheral basaloid cells, and a thin granular layer. Fibroblastic stroma and fronding of basaloid cells were also seen in both tumors. The trichoepitheliomas and milia differed mainly in the greater proportion of keratinized cells found in the milia. Clinically, the milia were found only in areas of vellus hairs. References 1. Hashimoto K, Lever WF: Tumors of the skin appendages , in Fitzpatrick TB, et al (eds): Dermatology in General Medicine . New York, McGraw-Hill Book Co Inc, 1971, pp 440-465. 2. Gray HR, Helwig EB: Epithelioma adenoides cysticum and solitary trichoepithelioma . Arch Dermatol 87:143-154, 1963.Crossref 3. Crain RC, Helwig EB: Dermal cylindroma (dermal eccrine cylindroma) . Am J Clin Pathol 35:504-515, 1961. 4. Adamson HG: On the nature of rodent ulcer . Lancet 1:810-811, 1914.Crossref 5. Cockayne EA: Inherited Abnormalities of the Skin and Its Appendages . London, Oxford University Press, 1933, p 394. 6. Whiffen JD: Dermal cylindroma . Plast Reconstr Surg 31:70-74, 1963.Crossref 7. Brooke HG: Epithelioma adenoides cysticum . Br J Dermatol 4:269-286, 1892. 8. McKusick VA: Mendelian Inheritance In Man . Baltimore, ed 3. Johns Hopkins University Press, 1971, p 91. 9. Welch JP, Wells RS, Kerr CB: Ancell-Spiegler cylindromas (turban tumors) and Brooke-Fordyce trichoepitheliomas: Evidence for a single genetic entity . J Med Genet 5:29-35, 1968.Crossref 10. Epstein W, Kligman AH: The pathogenesis of benign tumors of the skin . J Invest Dermatol 26:1-11, 1956.Crossref 11. Thies W, Schwarz E: Multiple eruptive milien-ein organoides follikhamartom . Archiv Klin Exp Dermatol 214:21-34, 1961.Crossref 12. Montagna W: The Structure and Function of the Skin , ed 2. New York, Academic Press Inc, 1962, pp 38-84.
Archives of Dermatology – American Medical Association
Published: May 1, 1975
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