Pyoderma gangrenosum as a cause of spontaneous vulvovaginal ulceration

Abstract

Pyoderma gangrenosum (PG) is a rare, destructive inflammatory disease in which a painful infiltration of the skin breaks down to a make an enlarging ulcer. The ulcers are usually characteristic with elevated and undermined borders and may be solitary or multiple. PG may present in association with systemic disease, such as inflammatory intestinal diseases, polyarthritis, gammopathy and others. The predilection sites are the lower extremities, buttocks and abdomen, but any area of the body may be involved. The female lower genital tract may also be affected (1–3). Therefore, this entity should be known by gynecologists. We report a case of vulvovaginal PG. A 55-year-old woman was admitted to the hospital with spontaneous ulcerations of the vulva, vagina and the right gluteal region that had developed during the preceding week. The ulcerations developed rapidly as painful redness and infiltrations of the skin, followed by necrotization. She stated that “pieces of the skin fell off,” resulting in deep and very painful ulcerations. Her general condition was otherwise unaffected, including no symptoms of bowel disorder. She did not use any drugs, including nonsteroid antiinflammatory drugs (NSAIDs) Examination revealed five deep ulcers located in the gluteal region, the vaginal introitus (Fig. 1), the vaginal wall (Fig. 2) and the cervix, with undermined borders, varying in size from 1 to 7 cm in diameter. Pyoderma gangrenosum ulcers of the vulva (the left labium minus and the posterior commissure) and the right gluteal region at the time of diagnosis. Note the undermined borders. Pyoderma gangrenosum ulcers of the anterior vaginal wall at the time of diagnosis. Biopsies from the ulcer margin showed nonspecific changes with ulceration and neutrophilic infiltration without certain signs of vasculitis. Immunohistochemical examination did not detect deposits of immunoglobulin complexes, fibrin or complement. Blood and urinalysis were normal except for an elevated sedimentation rate (81 mm), C-reactive protein (CRP) (114 mg/L) and P-potassium (4.4 mmol/L) and P-creatinine (99 µmol/L). Bacterial culture from the ulcers grew colonies of Escherichia coli, probably of no pathological significance. All other investigations, including P-antinuclear antibodies, P-ANCA immunofluorescence test, the tumor markers CEA and CA125, smear and chest X-ray were negative. A diagnosis of PG was made and treatment with cyclosporin A (Sandimmun Neoral®) 5 mg/kg body weight daily was started immediately as the only therapy. The therapeutic response was remarkable, resulting in a significant decline in inflammation within the first 24 h. After 1 week of treatment, the patient felt much better and the ulcers were healing. After 1 month of therapy the ulcers were partly healed, without signs of inflammation. After 2 months the ulcers were completely healed, just leaving some scars and a defect in the left labium minus that was caused by the biopsies. The cyclosporin A dosage was reduced stepwise and terminated 3.5 months after onset of the disease. At the 6-month follow-up there were no signs or symptoms of recurrence. We report this case to increase the attention regarding PG as a possible diagnosis when dealing with spontaneous ulcerations of the female genital area. The diagnosis may be easy, provided one is aware of this condition. The most important differential diagnoses are infectious ulcers, especially herpes simplex, syphilis and ulcus molle (chancroid), carcinomas and Bechet's disease. The clinical appearance of the ulcerations, the acute onset and the pain, combined with the absence of signs and symptoms of an infectious condition, will usually be a sufficient basis for the diagnosis. Infectious disease should be ruled out by means of adequate microbiological and serological tests. Histological examination is nonspecific as for ulcers of any cause and typically shows neutrophilic inflammation, edema, and thrombosis of small- and medium-sized vessels at the base of the ulcer (4). There are no specific laboratory markers for PG. Correct diagnosis is important for early therapeutic intervention, and also for avoiding unnecessary surgical procedures that may easily increase the ulcerations, especially in the acute phase of the disease. Cyclosporin A is usually very effective in the treatment of PG (5). The disease may relapse after termination of the immunosuppressive therapy, requiring sustained treatment, possibly for several years. As PG is associated with inflammatory bowel disease, these patients should be queried specifically with respect to these conditions.