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面部顽固性坏疽性脓皮病:一例报告

Recalcitrant Pyoderma Gangrenosum of the Face: A Case Report.

作者信息

Jiang Megan, Zhang Grace, Hsieh Tsung-Yen

机构信息

Otolaryngology, University of Cincinnati College of Medicine, Cincinnati, USA.

Otolaryngology, University of Cincinnati Medical Center, Cincinnati, USA.

出版信息

Cureus. 2024 Mar 28;16(3):e57136. doi: 10.7759/cureus.57136. eCollection 2024 Mar.

Abstract

Pyoderma gangrenosum (PG) is a rare autoinflammatory neutrophilic dermatosis. The ulcerative subtype presents with a tender nodule or pustule that progresses into a painful, necrotic ulcer.New lesions arise after minor trauma in one-third of patients, a phenomenon termed "pathergy." We present a 62-year-old Caucasian female with primary sclerosing cholangitis, hepatic cirrhosis, chronic hepatitis B, and severe PG. At the initial presentation, she had lesions on her face and four extremities. She had severe full-thickness ulcerations on the bilateral cheeks and underwent incision and drainage with washout of bilateral maxillary abscesses, left sinus curettage, and wound debridement. She has required multiple hospitalizations for severe flares. Treatment with steroids was complicated by spinal compression fractures. Steroid-sparring agents were ineffective. Her lesions involved bilateral cheeks, temples, temporal scalp, and eyelids with oroantral fistulae. Her facial ulcerations included a large septal perforation causing saddle nose deformity and eradication of a branch of the left facial nerve causing incomplete eye closure. She underwent bilateral facial wound irrigation with antibiotic irrigation and wound debridement. Due to social factors, she has been lost to follow-up and a definitive etiology of her PG has not yet been elucidated. Although rare, PG should remain a consideration in patients with ulcerative lesions on the head and neck. Wound debridement is typically discouraged given the risk of pathergy, but there may be a role for surgical intervention in adequately immunosuppressed patients.

摘要

坏疽性脓皮病(PG)是一种罕见的自身炎症性嗜中性皮肤病。溃疡性亚型表现为一个触痛性结节或脓疱,进而发展成一个疼痛的坏死性溃疡。三分之一的患者在轻微创伤后会出现新的皮损,这一现象被称为“同形反应”。我们报告一名62岁的白种女性,患有原发性硬化性胆管炎、肝硬化、慢性乙型肝炎和严重的PG。初诊时,她的面部和四肢有皮损。双侧脸颊有严重的全层溃疡,并接受了切开引流以及双侧上颌脓肿冲洗、左侧鼻窦刮除术和伤口清创术。她因严重病情发作多次住院治疗。使用类固醇治疗出现了脊柱压缩性骨折的并发症。类固醇替代药物无效。她的皮损累及双侧脸颊、颞部、颞顶头皮和眼睑,并伴有口鼻瘘。她的面部溃疡包括一个导致鞍鼻畸形的大鼻中隔穿孔,以及一条左侧面神经分支被破坏导致眼睑闭合不全。她接受了双侧面部伤口抗生素冲洗和伤口清创术。由于社会因素,她失访了,其PG的确切病因尚未阐明。尽管罕见,但PG仍应作为头颈部溃疡性皮损患者的一个考虑因素。鉴于存在同形反应的风险,通常不鼓励进行伤口清创,但在免疫抑制充分的患者中,手术干预可能会发挥作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8bed/11055618/4d3913723cda/cureus-0016-00000057136-i01.jpg

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