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Localized Birt-Hogg-Dubé Syndrome With Prominent Perivascular Fibromas

Schulz, Tilman M.D.; Ebschner, Ulrike M.D.; Hartschuh, Wolfgang M.D.

The American Journal of Dermatopathology: April 2001 - Volume 23 - Issue 2 - p 149-153
Extraordinary Case Reports

The autosomal dominant Birt-Hogg-Dubé syndrome is a cutaneo-intestinal condition that manifests on the skin in the form of multiple, skin-colored small papules that, histologically, prove to be mantleomas (fibrofolliculomas and trichodiscomas). These cutaneous lesions usually appear in the region of the head, neck, and upper part of the trunk. To date, only a single report in the literature describes the localized occurrence of this syndrome. We now describe a localized form of the Birt-Hogg-Dubé syndrome in a man with multiple mantleomas that were confined to the left half of the face, and which, in part, were arranged in the form of plaques. Another striking finding in this patient was a conspicuous vascular component in the lesions, characterized by a pronounced, well-demarcated fibrosis in the region of cutaneous blood vessel proliferations. Because perivascular fibromas have already been observed in other patients with Birt-Hogg-Dubé-syndrome, the perivascular fibroma, with fibrofolliculoma and trichodiscoma, must be included within this syndrome's spectrum of skin changes.

Institute of Pathology (Director: Prof. Dr. P.H. Wuensch), Nuremburg, Germany (T.S.); Department of Dermatology, University of Heidelberg, Heidelberg, Germany (U.E., W.H.).

Address correspondence and reprint requests to Prof. Dr. Wolfgang Hartschuh, Universitäts–Hautklinik, Vosstrasse 2, 69115 Heidelberg, Germany.

© 2001 Lippincott Williams & Wilkins, Inc.