Multiple familial trichoepitheliomas: A folliculosebaceous-apocrine genodermatosis

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We reviewed the pathologic findings on a family with multiple hereditary trichoepitheliomas. Although the majority of the lesions were trichoepitheliomas, basal cell carcinomas, spiradenomas, and spiradenomas with cylindromatous foci (spiradenocylindroma) were present, representing a spectrum of lesions exhibiting folliculosebaceous (trichoepithelioma, basal cell carcinoma) and apocrine (spiradenoma, spiradenocylindroma) differentiation. Multiple familial trichoepitheliomas may be a syndrome whereby tumors develop from undifferentiated germinative cells of the folliculosebaceous-apocrine unit. Published findings regarding the genetics of this syndrome and solitary trichoepitheliomas are reviewed; although the molecular basis for the tumors has yet to be determined, current data suggest that a tumor suppressor gene may be involved.

Original languageEnglish (US)
Pages (from-to)402-405
Number of pages4
JournalAmerican Journal of Dermatopathology
Issue number5
StatePublished - Oct 1 2002

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Dermatology


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