Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that most often presents with painful ulcerations of violaceous borders in lower limbs and/or trunk. PG treatment varies according to the severity of the lesion and may either respond to local therapies or require immunosuppressive agents. In this article, we present the case of an antiphospholipid antibody-positive 59-year-old female patient diagnosed with granulomatosis with polyangiitis who developed severe PG-like skin involvement that was responsive to rituximab therapy.
Tashtoush B, Memarpour R, Johnston Y, Ramirez J. Large pyoderma gangrenosum-like ulcers: a rare presentation of granulomatosis with polyangiitis. Case Rep Rheumatol 2014;2014:850364.
Ahronowitz I, Harp J, Shinkai K. Etiology and management of pyoderma gangrenosum: a comprehensive review. Am J Clin Dermatol 2012;13:191-211.
Sen M, Dogra S, Rathi M, Sharma A. Successful treatment of large refractory pyoderma gangrenosumlike presentation of granulomatosis with polyangiitis by rituximab. Int J Rheum Dis 2017;20:2200-2.
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65:1-11.
Rees JD, Lança S, Marques PV, Gómez-Puerta JA, Moco R, Oliveri C, et al. Prevalence of the antiphospholipid syndrome in primary systemic vasculitis. Ann Rheum Dis 2006;65:109-11.
Jordan N, D’cruz DP. Association of lupus anticoagulant with long-term damage accrual in antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res (Hoboken) 2016;68:711-5.
Lally L, Sammaritano LR. Vasculitis in antiphospholipid syndrome. Rheum Dis Clin North Am 2015;41:109-23.
de Cambourg G, Mahé A, Banea S, Moulinas C, Blaison G. Ganulomatosis with polyangiitis revealed by a cutaneous ulceration mimicking a pyoderma gangrenosum: Two patients. Rev Med Interne 2016;37:632-5.
Donmez S, Pamuk ON, Gedik M, A K R, Bulut G. A case of granulomatosis with polyangiitis and pyoderma gangrenosum successfully treated with infliximab and rituximab. Int J Rheum Dis 2014;17:471-5.
Tenedios F, Erkan D, Lockshin MD. Rituximab in the primary antiphospholipid syndrome (PAPS) Arthritis Rheum 2005;52:4078.
Erkan D, Vega J, Ramón G, Kozora E, Lockshin MD. A pilot open-label phase II trial of rituximab for noncriteria manifestations of antiphospholipid syndrome. Arthritis Rheum 2013;65:464-71.
Kindle S, Fanciullo J. Healing of leg ulcers associated with granulomatosis with polyangiitis (Wegener granulomatosis) after rituximab therapy. Cutis 2017;99:12-5.
Murthy RK, Jackson J, Chatham WW, Sami N. Extensive pyoderma gangrenosum associated with granulomatosis with polyangiitis with both responsive to rituximab. J Clin Rheumatol 2016;22:393-5.