Vulvar pyoderma gangrenosum with renal involvement.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis of unknown etiology which usually occurs over the lower extremities; however, unusual presentations such as that involving the genital region have been described. Extracutaneous involvement of PG in the form of sterile neutrophilic infiltrates in various organs has infrequently been reported. We hereby describe a case of PG that was limited to the vulvar and perianal area in a 37-year-old female, with associated renal involvement in the form of a slight increase in the serum creatinine, microhematuria of glomerular origin and proteinuria. The patient had a rapid response of both her mucocutaneous lesions and renal dysfunction after the initiation of systemic steroids. The present case highlights the importance of evaluating all patients with PG for extracutaneous disease to avoid potentially harmful diagnostic or therapeutic procedures. Two other reasons for interest are the localized presentation of disease on the genital region and the presence of vascular involvement, albeit without signs of true vasculitis, vascular changes possibly being a histological hallmark of PG involving genitalia.