MULTIPLE LIPOMAS, ANGIOLIPOMAS, AND PARATHYROID ADENOMAS IN A PATIENTWITH BIRT-HOGG-DUBE SYNDROME

A 60-year-old man presented to our clinic with a 40-year history of ma ny asymptomatic papules on the face, neck: and upper trunk (Fig. 1). T hese tumors occurred in association with acrochordon-like papules on t he neck and in the axillae. He also had numerous tumors, many of which were painful, on the trunk, buttocks, and extremities; the biopsies o f many of them showed lipomas and angiolipomas. These tumors started i n his early twenties and have continued to increase in number and size . Past medical history was remarkable for multiple spontaneous pneumot horaces starting at 15 years of age, bullous emphysema in his early tw enties, parathyroid adenoma at 50 years of age, and prostatic adenocar cinoma at 47 years of age. The family history was remarkable for two d aughters with similar facial papules that had first appeared at about 30 years of age, The daughters were not available for examination. The patient had multiple 3 to 5 mm, skin-colored to white, smooth, dome-s haped papules on the face, neck, and upper trunk (Fig. 1). He had mult iple soft, flesh-colored, pedunculated papules, consistent with acroch ordons, in the axillae and on the sides of the neck, He also had numer ous painful and painless soft nodules and tumors, consistent with lipo mas and angiolipomas, on the trunk and extremities. Histopathologic ex amination of a facial papule showed multiple cystically dilated hair f ollicles, each of which was encircled by a fibromucinous proliferation of the dermis with loose fibrillary collagen bundles (Pig. 2). Most o f the cystic follicles had angular projections of the epithelium exten ding into the surrounding fibrillary collagen stroma. These features a re consistent with fibrofolliculoma. There were also clusters of elong ated sebaceous lobules (Pig. 3) at the periphery of the altered stroma , more characteristically seen in trichodiscomas, within the same biop sy specimen. Acrochordon-like lesions were not biopsied.