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

Abstract

Systemic autoimmune diseases are characterized by a wide variety of clinical manifestations, including cutaneous lesions. Pyoderma gangrenosum (PG) and pyodermalike lesions can be associated with different rheumatic conditions, such as granulomatosis with polyangiitis (GPA). 1 Management of this pathology is based on local wound care, avoidance of trauma, and in some occasions, systemic therapy with systemic corticosteroids and cyclosporine being the mainstays of treatment. In recalcitrant PG, biologic medications, such as tumor necrosis factor-alpha (TNF-a) inhibitors, could be necessary. Actually, infliximab (IFX) is the only biologic that has shown efficacy in classic PG in a randomized, double-blind, controlled trial (level 1 evidence). 2 Rituximab (RTX) was approved for use in GPA by the Food and Drug Administration in April 2011. The use of RTX for cutaneous and subcutaneous GPA lesions has previously been reported in some case series and case reports. 3 In this article, we present the case of an antiphospholipid antibody (aPL)-positive female patient diagnosed with GPA who developed severe PG-like skin involvement that was responsive to RTX therapy.