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Three patients had hereditary multiple trichodiscomas of early onset and dominant inheritance. This report is an analysis of the histologic, histochemical, immunohistochemical, and electron microscopic findings in 27 trichodiscomas, the largest published series so far, to our knowledge. The tumors of perifollicular connective tissue are briefly reviewed and a classification is proposed. Hereditary multiple trichodiscomas are regarded as a new entity that should be distinguished from the syndrome recently described by Birt and associates that is characterized by autosomal dominant inherited fibrofolliculomas as the hallmark, associated with trichodiscomas and acrochordons.

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