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Peyronie syndrome

Orpha number ORPHA2870
Prevalence of rare diseases >1 / 1000
Inheritance
  • Sporadic
  • Autosomal dominant
Age of onset Adulthood
ICD 10 code
  • N48.6
MIM number
Synonym(s) -

Summary

Peyronie's disease (PD) has been described for the first time in 1743 by François de La Peyronie. It is a connective-tissue disorder affecting the tunica albuginea of the corpora cavernosa. After an initial inflammatory stage, the cellular infiltrate is gradually replaced by a focal fibrosis, rich in collagen type I and II, and resulting in the formation of plaques, with loss of elasticity and penile deformation during erection. Clinical signs associate variable degrees of paraesthesias, painful erection, penile curvature, penile rigidity and erectile dysfunction. Exact pathogenesis remains unknown. Frequent association with HLA B27 antibody evokes an infectious etiology leading to an autoimmune cross-reaction. The role of repeated penile micro-traumatisms during intercourse appears to favor the onset of inflammatory phenomena. Immunological hypothesis suggests an auto-immune alteration of conjunctive tissue, similar to that involved in the genesis of other connective tissue disorders. The diagnosis is based mainly on the clinical picture dominated by erectile dysfunction. Penile echography or magnetic resonance imaging can supplement morphological evaluation of plaques and detect the presence of calcifications. Annual incidence of the PD varies between 0.3 and 3% with a prevalence of 388 cases per 100,000 inhabitants. Rare before 40 years old, its frequency gradually increases with a peak around the age of 60. Although benign, the functional prognosis of the PD remains poor. The surgical techniques, seeking to correct the penile deformation, lead to satisfactory results in about 75% of cases. Other conservative therapeutic means, like vitamin E therapy and local corticoid injections, seems to have encouraging results. *Author: Orphanet (January 2004).*

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