Multiple facial angiofibromas: A cutaneous manifestation of Birt-Hogg-Dubé syndrome - 21/08/11
, Mona A. Gohara, MD, Jennifer M. McNiff, MD, Sumaira Z. Aasi, MD, Israel Dvoretzky, MD
Reprint requests: Julie Schaffer, MD, Department of Dermatology, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06520.New Haven, Connecticut
Abstract |
Birt-Hogg-Dubé syndrome (BHDS) is an uncommon autosomal dominant genodermatosis characterized by a triad of skin tumors—fibrofolliculomas, trichodiscomas, and acrochordons—together with an increased risk of renal tumors and spontaneous pneumothoraces. This report describes multiple facial angiofibromas as the predominant initial manifestation of BHDS. The patient had a total of 41 facial papules removed via shave excision, initially for diagnostic and then for therapeutic purposes; histologic evaluation revealed diagnostic features of angiofibroma in 39 lesions and fibrofolliculoma in only 2. BHDS should be considered, along with tuberous sclerosis and multiple endocrine neoplasia type 1, in the differential diagnosis of multiple facial angiofibromas, particularly when onset is in adulthood.
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| Supported by Stiefel Laboratories. Funding sources: None. Conflicts of interest: None identified. Presented at a meeting of the New England Dermatological Society at Yale University on October 25, 2003. |
Vol 53 - N° 2S
P. S108-S111 - août 2005 Retour au numéro
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