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September 1997

Multiple, Hereditary Dome-Shaped Papules and Acrochordons

Author Affiliations

Hospital of the University of Pennsylvania, Philadelphia (Drs Haimovitz, Halpern, and Heymann), and the University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School, Camden (Dr Heymann)

Arch Dermatol. 1997;133(9):1166. doi:10.1001/archderm.1997.03890450117018

REPORT OF A CASE  A 42-year-old white man presented for evaluation of numerous asymptomatic papules and skin tags that had developed decades earlier. In his teens, he noted the onset of multiple skin tags (Figure 1). In his early 20s, he developed innumerable 2- to 5-mm, pale, yellow-brown, dome-shaped papules, predominantly on the face, chest, and upper back area (Figure 2). There were no significant oral lesions. Close inspection of the numerous papules revealed that some were follicular, while others demonstrated a hair toward the periphery of a given papule. The patient reported that his father, paternal grandfather, uncle, and aunt had skin lesions that were similar to his own. The patient's only child, a 6-year-old son, was free of skin disease.Skin biopsy specimens of 2 papules are shown in Figure 3 and Figure 4.What is your diagnosis?

DIAGNOSIS:  Birt-Hogg-Dubé syndrome.