Peyronie Disease

Clinical Features

Penile contracture is characterized by the formation of thickened fibrous plaques on the dorsum of the penis. The condition bears certain fundamental similarities to Dupuytren contracture of the hand and the 2 occur rather frequently in the same subject. Peyronie disease has been induced by adrenergic blockers such as propranolol and practolol (Kristensen, 1979).

In Rome, Carrieri et al. (1998) performed a case-control study of 134 men with Peyronie disease and 134 male controls. Men who had undergone invasive procedures on the penis (e.g., urethral catheterization, cystoscopy, and transurethral prostatectomy) had a 16-fold increased risk of Peyronie disease, while a nearly 3-fold increase was observed among men who had genital and/or perineal trauma. A history of urethritis, uricacidemia, and lipoma was also significantly associated with increased risk for Peyronie disease. Dupuytren contracture was found in 21% of cases and none of the controls, and 4% of the cases and none of the controls reported familial history for Peyronie disease. The frequency of inflammatory or fibromatous lesions of the genital tract of the partner were significantly higher in men with Peyronie disease than among controls. These results were consistent when performing a stratified analysis according to the type of controls (i.e., controls affected by urologic or by digestive conditions) to rule out the potential effect of recall bias. The importance of genital trauma and genetic background in the development of Peyronie disease was supported.

Bivalacqua et al. (2000) reviewed published research on the pathophysiology of Peyronie disease; they concluded that the lesion starts as a sclerosing inflammatory process and develops into a connective tissue disorder involving the tunica albuginea, but noted that the etiology remains poorly understood.

Inheritance

An anonymous nongeneticist suggested to McKusick (1980) that Peyronie disease is 'sex-linked with reduced penetrance.' Bias et al. (1982) concluded that this phenotype is a male-limited, autosomal dominant trait. They traced Peyronie disease through several families. Dupuytren contracture was often present in both males and females. In 1 kindred, males in 3 successive generations had Peyronie disease and Dupuytren contractures, and the latter was present in a fourth generation. Close linkage with HLA (see 142800) was excluded. Chromosomal abnormalities were described by Somers et al. (1987) and by Guerneri et al. (1991).

History

Musitelli et al. (2008) reviewed historical reports from as far back as the 13th century for what have been described by modern medical historians as possible cases of Peyronie disease, but concluded that the first real description was that of De La Peyronie himself, who described 2 men, 30 years and 40 years old, respectively, with 'indurations of the cavernous bodies' of the penis (De La Peyronie, 1743).