Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands - A report of 7 cases and review of the literature
Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands - A report of 7 cases and review of the literature
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DOI：
10.1001/archderm.138.3.361
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发表时间：
2002-03-01
影响因子：
--
通讯作者：
Connolly, SM
中科院分区：
文献类型：
--
作者：
DiCaudo, DJ;Connolly, SM
背景：手背中性粒细胞皮肤病（脓疱性血管炎）是最近发现的一种疾病，临床上可能类似于Sweet综合征的局部变异。目的：描述这种罕见疾病的临床和组织病理学特征；将这些特征与Sweet综合征进行比较和对比；并调查其与全身性疾病的关系。观察：7名妇女被转诊为脓疱或溃疡斑块和结节在手背。在大多数患者中，最初诊断为皮肤感染，但抗生素治疗无效。皮肤活检标本显示密集的真皮中性粒细胞浸润，伴白细胞减少和纤维蛋白样血管坏死。所有病例的皮肤培养结果均为阴性。强的松和氨苯砜似乎有帮助，但复发是常见的。盐酸米诺环素的疗效不确定。在7例患者中，可能的系统性关联包括肠道疾病和尿路感染。结论：手背中性粒细胞性皮肤病可能与Sweet综合征密切相关，但常表现为白细胞分裂性血管炎的组织学模式。认识到这种疾病是很重要的，因为它可能被误诊为局部皮肤感染。需要进一步的研究来进一步调查其与内部疾病，特别是肠道疾病的可能关联。
Background: Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands is a recently described disorder, which may clinically resemble a localized variant of Sweet syndrome.Objectives: To describe the clinical and histopathologic characteristics of this rare disorder; to compare and contrast these features with those of Sweet syndrome; and to investigate possible associations with systemic diseases.Observations: Seven women were referred for pustular or ulcerative plaques and nodules on the dorsal hands. In most patients, the initial diagnosis was cutaneous infection, but antibiotic therapy was ineffective. Skin biopsy specimens showed dense dermal neutrophilic infiltrates with leukocytoclasis and fibrinoid vascular necrosis. Cutaneous cultures yielded negative findings in all cases. Prednisone and dapsone appeared to be helpful, but recurrences were common. Minocycline hydrochloride was of uncertain benefit. Among the 7 patients, possible systemic associations included bowel disorders and a urinary tract infection.Conclusions: Neutrophilic dermatosis of the dorsal hands may be closely related to Sweet syndrome but frequently shows the histologic pattern of leukocytoclastic vasculitis. Recognition of this disorder is important, because it may be misdiagnosed as a localized cutaneous infection. Additional studies are needed to investigate further the possible associations with internal diseases, especially bowel disorders.