Silva Cristina, Freitas Sara, Costa Ana, Alves Glória, Cotter Jorge
Internal Medicine, Hospital Senhora da Oliveira, Guimarães, PRT.
Cureus. 2021 Oct 7;13(10):e18581. doi: 10.7759/cureus.18581. eCollection 2021 Oct.
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.
嗜酸性肉芽肿性多血管炎（EGPA）是一种罕见的系统性血管炎。本报告描述了一名68岁女性的病例，该患者因广泛的出血性大疱性病变就诊于急诊科，病变累及肘部、手背、足部和膝盖，伴有组织缺失和坏死区域。评估结果诊断为抗中性粒细胞胞浆抗体阳性的EGPA，累及多系统：皮肤、肺、肾、肠道以及外周和中枢神经系统。她接受了糖皮质激素和静脉注射免疫球蛋白治疗。她出现了多种多重耐药菌感染并发症。诊断两个月后，患者无呼吸或胃肠道体征或症状，蛋白尿较轻。然而，她仍有广泛的溃疡，并患有使人致残的感觉异常。在所有感染得到控制后，我们决定开始使用利妥昔单抗治疗。她还接受了切除清创和异体移植物修复，后来又接受了肘部和足部的自体移植物修复。她的临床反应良好，伤口完全愈合。本病例旨在说明一种严重的EGPA形式，伴有严重的多系统受累，导致了较高的发病率。在平衡患者对免疫抑制治疗的需求与高感染风险之间是一项临床挑战。尽管如此，我们认为疾病控制对于皮肤恢复、更好的身体康复和更好的生活质量至关重要。
