BACKGROUND: Brooke-Spiegler syndrome is a hereditary tumor predisposition disorder characterized by the development of cylindromas, trichoepitheliomas, and spiradenomas. Predilection sites of the disease are hair follicles and sweat glands of the head and neck. In some patients, the tumors can coalesce to so-called turban tumors, which then usually cause cosmetic, psychological, and functional impairment. A curative therapy is not yet available, and thus total scalp excision followed by split skin graft is evolving as a frequently applied therapy. However, this treatment can lead to the formation of a thin and vulnerable skin, which hampers wearing a wig. Therefore, a more robust and functional solution is preferable. Here, we report on a woman with a turban tumor who suffered enormously from the disease and had secluded herself from social life. METHODS: We treated her with a total scalp excision down to the periosteum, followed by sequential combined reconstruction with an artificial dermal template and split skin grafts. RESULTS: The treatment resulted in formation of a robust and flexible skin. CONCLUSION: Treatment of turban tumor is a challenge considering the localization and extensiveness of the tumor masses. This novel therapy for turban tumor leads to a very good cosmetic and functional outcome.
- FAMILIAL CYLINDROMATOSIS