LAUSR

Dear Editor, Skin involvement is seen in almost half of patients with granulomatosis with polyangiitis (GPA), an antineutrophil cytoplasmic antibodies (ANCA)associated vasculitis (AAV), and these may be the presenting complaint in 10–25% of cases. AAV may affect both small and medium-sized blood vessels, which results in varied cutaneous presentations (i.e., urticarial lesions, palpable purpura with small vessel involvement and nodules, ulcers, livedo racemosa with medium vessel involvement). However, the spectrum of cutaneous involvement varies widely, and these may resemble other diseases like polyarteritis nodosa and pyoderma gangrenosum (PG). The PG-like lesions can occur over lower extremities, face, trunk and arms, and have been reported in up to 27% of GPA patients having skin involvement. Dermatologic manifestations have been associated with a higher frequency of joint and renal involvement and are usually responsive to immunosuppression with steroids and cyclophosphamide given for management of systemic manifestations of GPA; however, some may be refractory. Herein, we present a case of a 29-year-old woman suffering from limited GPA with a large non-healing vasculitic ulcer over the thigh which was refractory to systemic corticosteroids, methotrexate, mycophenolate mofetil and cyclophosphamide and eventually responded only to rituximab.