Trichoepitheliomas, Multiple Desmoplastic

Desmoplastic trichoepithelioma is a benign neoplasm with follicular differentiation. It was described as a distinctive clinicopathologic entity by Brownstein and Shapiro (1976, 1977) and MacDonald et al. (1977). Brownstein and Shapiro (1976) described desmoplastic trichoepitheliomas as 'a variant of solitary trichoepithelioma with a distinctive annular configuration and sclerotic consistency clinically.' They predicted that a form of multiple desmoplastic trichoepitheliomas inherited in an autosomal dominant fashion would eventually be discovered. Shapiro and Kopf (1991) described such a family. Grandmother, daughter, and son were affected. Dervan et al. (1985) described familial solitary desmoplastic trichoepitheliomas. This disorder is distinct from the inherited syndrome of multiple trichoepitheliomas, also referred to as epithelioma adenoides cysticum of Brooke (601606).