Eosinophilic Granulomatosis With Polyangiitis With Extensive Cutaneous Involvement

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis. This report describes the case of a 68-year-old female that showed up at the emergency department with extensive haemorrhagic bullous lesions, affecting elbows, the dorsal side of hands, feet and knees, with loss of tissue and necrotic areas. The evaluation led to the diagnosis of antineutrophil cytoplasmic antibody-positive EGPA with multisystem involvement: cutaneous, pulmonary, renal, intestinal and peripheral and central nervous system. She received corticosteroids and intravenous immunoglobulin. She developed multiple infectious complications with multidrug-resistant bacteria. Two months after the diagnosis, the patient had no respiratory or gastrointestinal signs or symptoms, and the proteinuria was mild. Yet, she maintained extensive ulcers and was suffering from disabling dysesthesias. After the resolution of all infections, we decided to start rituximab. She was also submitted to excisional debridement and heterologous graft repair and later to autologous graft repair of elbows and feet. She had a good clinical response with complete healing of the wounds. This case intends to illustrate a serious form of EGPA, with severe multisystem involvement that resulted in great morbidity. It was a clinical challenge to balance the need for immunosuppressive therapy with the high infectious risk of the patient. Nonetheless, we considered that disease control was fundamental to skin recovery, better physical rehabilitation and better quality of life.