A RARE ASSOCIATION OF MULTIPLE HEREDITARY TRICHOEPITHELIOMAS WITH MULTIPLE SPIRADENOMAS

We report on a 49-year-old woman and her 20-year-old daughter with mul tiple papular and nodular lesions on the face and scalp, showing the t ypical clinical and histopathological features of trichoepithelioma. I n addition, the mother had developed multiple, blue-coloured and painf ul lesions on her breast and back over the previous eight years, showi ng the histopathological characteristics of eccrine spiradenomas. In a tissue specimen both trichoepitheliomas and spiradenomas were found, in close proximity to each other. A coexistence of trichoepitheliomas and cylindromas has been described repeatedly, whereas only three case s of trichoepitheliomas associated with spiradenomas have so far been reported in the literature. Spiradenoma is a rare and mostly solitary adnexal tumour and the occurrence of multiple spiradenomas as describe d in our patient is an unusual feature. Interestingly, in all three re ported cases of trichoepithelioma associated with spiradenoma, multipl e tumours of both types occurred. The association of multiple heredita ry trichoepitheliomas with multiple spiradenomas, localized partly in close proximity is a rare clinical feature, suggesting a common develo pmental defect.