Pyoderma gangrenosum of the vulva treated with mycophenolate mofetil and infliximab

Abstract

Dear Editor, Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by chronic, painful, recurrent ulcerations with erythematous, rolled, undermined borders. PG most commonly occurs on the lower extremities but can affect any mucocutaneous site. Diagnosis is often delayed due to its rarity and unknown etiology; PG is characteristically considered when ulcers fail to heal or worsen after surgical debridement. We report the case of a 30-year-old woman who presented for evaluation of chronic vulvar ulcerations with associated pain, bleeding, pruritus, and dyspareunia for 4 years. Gynecology had previously diagnosed and treated as plasma cell vulvitis. The patient experienced mild improvement with topical and intralesional corticosteroids over 2 years. She subsequently underwent surgical excision of the bilateral vaginal vestibule complicated by postoperative failure to heal and ulcer recurrence. Persistent disease caused significant functional impairment. Physical examination demonstrated coalescing, erythematous, friable, exophytic papules with ragged, actively bleeding, superficial ulcerations involving the bilateral and posterior vaginal introitus (Figure 1a). Ulcer borders were violaceous, rolled, and edematous. Vulvar biopsy exhibited chronic suppurative and granulomatous mucositis with dense plasma cell infiltrates (Figure 2). Kappa and lambda immunostains demonstrated a polyclonal process. Microbiologic (Warthin–Starry, Grocott–Gomori methenamine silver, acid-fast bacilli) stains and tissue cultures (bacteria, fungi, mycobacteria) were negative. Evaluations for associated infection (HBV, HCV, HIV, tuberculosis, syphilis), autoimmune disease, malignancy, or inflammatory bowel disease, including colonoscopy, were negative. Based on clinico-histopathological findings, vulvar PG was diagnosed. Prednisone 60 mg (1 mg/kg) daily was initiated, and ulcers healed completely within 4 weeks. Prednisone was gradually tapered; ulcers recurred at 30 mg daily. Mycophenolate mofetil was instituted as steroidsparing therapy (initially 500 mg twice daily with gradual increase to 1,500 mg twice daily) with concomitant prednisone taper (Figure 1b). Halobetasol 0.05% ointment was also applied once daily. Disease control was maintained on mycophenolate and halobetasol ointment for 1 year when preconception planning warranted transition to infliximab (5 mg/kg). The patient has since successfully conceived, carried to term, and delivered via elective cesarean section two healthy baby boys while on infliximab. She is currently pregnant and receiving infliximab infusions every 7 weeks. Pyoderma gangrenosum is a diagnosis of exclusion. For vulvovaginal ulcerations, the more common etiologies of infection, aphthae, and vulvar intraepithelial neoplasia/squamous cell carcinoma must be also considered. Histopathology, tissue culture, and comprehensive laboratory evaluation help to exclude other etiologies. Approximately 50% of patients with PG have an associated autoimmune or hematologic disease, malignancy, or inflammatory bowel disease (Ashchyan et al., 2018); therefore, it is imperative to conduct