PEYRONIES DISEASE - A NEWLY RECOGNIZED AUTOSOMAL-DOMINANT TRAIT

Peyronie''s disease, a connective tissue disorder of unknown cause, is characterized by the formation of thickened fibrous plaques on the dorsum of the penis. It often occurs simultaneously with other fibrotic changes, most notably Dupuytren''s contracture of the hands or feet. No previous reports suggested inheritance of this syndrome; however, Willscher et al. reported an association between Peyronie''s disease and antigens of the HLA-B7 cross-reacting group. Family studies were undertaken when 3 patients reported similarly affected 1st-degree relatives. One kindred showed father-to-son transmission of Peyronie''s disease with Dupuytren''s contracture in 3 generations. Pedigree analysis of the 3 families suggests that Peyronie''s syndrome is a male-limited, autosomal-dominant trait. Antigens of the HLA-B7 cross-reacting group occurred in all 3 kindreds; however, the data ruled out close linkage of the disease and HLA.