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手背中性粒细胞皮肤病:123 例病例回顾。

Neutrophilic dermatosis of the dorsal hands: A review of 123 cases.

机构信息

Department of Dermatology, Sir Paul Boffa Hospital, Floriana, Malta.

Department of Dermatology, Sir Paul Boffa Hospital, Floriana, Malta.

出版信息

J Am Acad Dermatol. 2023 Jun;88(6):1338-1344. doi: 10.1016/j.jaad.2019.08.070. Epub 2019 Sep 6.

DOI:10.1016/j.jaad.2019.08.070
PMID:31499154
Abstract

Neutrophilic dermatosis of the dorsal hands (NDDH) is an uncommon localized variant of Sweet syndrome first described in 1995. It is characterized by tender erythematous plaques, pustules, and bullae on the dorsa of the hands. A total of 123 cases of NDDH are included in this review. The mean patient age was 62.1 years, and there was a slight female preponderance. Overall, 78.0% of cases had bilateral involvement, and other sites were affected in almost a third of cases. Underlying disease was found in ∼40% of patients, with the most common associations being hematologic disorders (gammopathies, myelodysplasias, or malignancies), recent infection, solid organ tumors, and inflammatory bowel disease. Systemic or topical corticosteroids or both were employed in the treatment of 88.1% of cases, while dapsone, colchicine, and tetracyclines were the most common steroid-sparing agents used. Improvement was often rapid and complete resolution the norm. Although uncommon, NDDH is frequently misdiagnosed, and thus, its exact prevalence is probably underestimated. Misdiagnosis might have significant implications, including treatment delays or incorrect management. Moreover, recognition of NDDH is important, since a correct diagnosis should trigger a search for underlying diseases and proper treatment with corticosteroids, steroid-sparing agents, or both, which is almost invariably curative.

摘要

中性粒细胞性皮肤病的手背 (NDDH) 是 Sweet 综合征的一种罕见局部变异型,于 1995 年首次描述。其特征是在手背出现疼痛性红斑、脓疱和大疱。本综述共纳入 123 例 NDDH 病例。患者平均年龄为 62.1 岁,女性略多。总体而言,78.0%的病例为双侧受累,近三分之一的病例累及其他部位。约 40%的患者存在基础疾病,最常见的关联是血液系统疾病(单克隆丙种球蛋白病、骨髓增生异常或恶性肿瘤)、近期感染、实体器官肿瘤和炎症性肠病。88.1%的病例采用全身或局部皮质类固醇治疗,而氨苯砜、秋水仙碱和四环素是最常用的皮质类固醇节约剂。改善通常迅速且完全缓解是常态。尽管不常见,但 NDDH 经常被误诊,因此其确切患病率可能被低估。误诊可能有重大影响,包括治疗延迟或管理不当。此外,识别 NDDH 很重要,因为正确的诊断应引发对潜在疾病的搜索,并采用皮质类固醇、皮质类固醇节约剂或两者联合进行适当治疗,几乎总是可以治愈。

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