Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis

Abstract

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

Efficacy of Rituximab for Refractory Pyoderma Gangrenosum-Like Ulcers in Granulomatosis With Polyangiitis Associated to Antiphospholipid Antibodies

“…Other authors have reported similar findings with RTX in patients with PG or PG-like lesions who had responded unsuccessfully to corticosteroids 12,13 and immunosuppressive agents (Table 1). 1,3 In conclusion, this case shows that accurate diagnosis sometimes remains difficult, particularly in the presence of overlapping autoimmune diseases. This is when histopathological…”

“…Less frequently, articular and cutaneous involvement is seen. 1 Initially, our patient suffered polyarthritis indistinguishable from rheumatoid arthritis. However, during the course of her illness, she developed other clinical features (involvement of the respiratory tract and nervous system) that, along with positive serology tests for ANCA and anti-PR3, helped to diagnose GPA.…”

Granulomatosis with polyangiitis preceded with pyoderma gangrenosum in 41-year-old man

“…6 Difficulties in assessing the histopathological picture such as lack of clear diagnos� tic criteria for PG and recommendation to repeat biopsy in order to determine the proper diagnosis. 2,5,7 Therefore, epidemiological data regarding the actual co�e�istence of PG and PGA are controversial. 9,11 Genoveese et al 9 re� viewed 28 cases and established guidelines useful for dis� tinguishing these conditions: �1� typical raised, erythema� tous�wavy edges are less visible in GPA ulcers compared to PG; (2) histology of early PG lesion consists mainly of neutrophilic inflammatory infiltrate; (3) c-ANCA positiv� ity is compatible with GPA; (4) systemic involvement is common in GPA and rare in PG.…”

“…The most common skin lesions are palpable purpura, necrotic ulcera� tions, papules and nodules with many histological patterns: leukocytoclastic vasculitis, granulomatous vasculitis, and palisading granulomas. 2,3 The prevalence of GPA in Europe is 2.1-14.4 cases per million population per year. 4 Skin lesions in the course of GPA are not characteristic symptoms, but may be present in as many as 15%-50% of cases of the disease and suggest cutaneous GPA as in the case described below.…”

Establishing the Feasibility of Face Transplantation in Granulomatosis With Polyangiitis

“…Skin involvement in GPA can manifest as PG . PG can also be triggered by trauma or surgery (pathergy) .…”