Department of Dermatology, Faculty of Medicine, Zagazig University, Zagazig, Egypt.
Member of Interactive Dermatology Foundation Research group.
J Dtsch Dermatol Ges. 2021 Aug;19(8):1133-1143. doi: 10.1111/ddg.14449. Epub 2021 May 25.
Classic erythema nodosum leprosum (ENL) is characterized clinically by abrupt eruption of tender erythematous nodules, papules and plaques. Variable atypical patterns have been described, for example pustular, bullous, ulcerative, necrotic and Sweet's syndrome-like ENL. We aim to review previously reported cases of atypical ENL addressing the diagnostic and therapeutic aspects of these uncommon presentations. A search of medical literature for all cases of atypical ENL was conducted in the PubMed database till 2020. Data of patients with atypical ENL were collected and analyzed to describe the epidemiological, clinico-histological and therapeutic features. The major five clinically described presentations of atypical ENL include vesiculo-bullous lesions (46 % of patients), ulcero-necrotic lesions (41 %), erythema multiforme-like lesions (28 %), Sweet's syndrome-like lesions (11 %) and pustules (9 %). The skin lesions were accompanied by fever and constitutional symptoms in all patients. Oral steroids and thalidomide were the main lines of therapy in most of the reported patients. Dermatologists and pathologists should keep in mind the clinical variability of ENL to avoid misdiagnosis and delayed management. Early recognition can help control disease progression and save the patients from further complications.
经典结节性红斑麻风（ENL）临床上表现为突然出现的触痛性红斑结节、丘疹和斑块。已经描述了各种非典型表现，例如脓疱性、大疱性、溃疡性、坏死性和类Sweet 综合征的 ENL。我们旨在回顾先前报道的非典型 ENL 病例，以探讨这些不常见表现的诊断和治疗方面。在 PubMed 数据库中对所有非典型 ENL 病例进行了医学文献检索，检索时间截止到 2020 年。收集和分析了非典型 ENL 患者的数据，以描述其流行病学、临床组织学和治疗特征。非典型 ENL 的主要五种临床描述表现包括水疱大疱性病变（46%的患者）、溃疡性坏死性病变（41%）、多形红斑样病变（28%）、类 Sweet 综合征病变（11%）和脓疱（9%）。所有患者的皮肤病变均伴有发热和全身症状。大多数报道的患者主要采用口服类固醇和沙利度胺进行治疗。皮肤科医生和病理学家应牢记 ENL 的临床变异性，以避免误诊和延迟治疗。早期识别有助于控制疾病进展并避免患者出现进一步的并发症。
