Neutrophilic dermatosis of the dorsal hands: Pustular vasculitis revisited
Neutrophilic dermatosis of the dorsal hands: Pustular vasculitis revisited
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DOI：
10.1067/mjd.2000.109286
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发表时间：
2000-11-01
影响因子：
13.8
通讯作者：
James, WD
中科院分区：
文献类型：
--
作者：
Galaria, NA;Junkins-Hopkins, JM;James, WD
最近描述了一种称为“手部脓疱性血管炎”的实体。这种疾病的患者表现为低热和局限于手背和手指的肿胀性斑块、脓疱和大疱，其组织学特征为致密的嗜中性粒细胞浸润和白细胞破碎性血管炎。我们描述的患者有类似的临床表现，但谁没有血管炎的活检结果。我们描述了3例无症状的出血性大疱、斑块和脓疱患者，仅发生在手背上。活检标本显示嗜中性粒细胞浸润和白细胞破碎，但没有坏死性血管炎，并使人联想到斯威特的嗜中性粒细胞皮肤病。在我们的患者中，皮质类固醇或氨苯砜导致病灶清除，小剂量的氨苯砜维持治疗可防止复发。我们的3例患者的临床病变类似于那些被称为脓疱性血管炎的手，但缺乏白细胞破碎性血管炎的活检结果。由于组织学的发现和治疗反应更具有Sweet综合征的特征，我们建议将手背嗜中性皮肤病命名为手背嗜中性皮肤病。此外，低剂量氨苯砜被建议作为这种情况下，特别是在那些复发性疾病的可能的一线治疗。
An entity termed "pustular vasculitis of the hands" was recently described. Patients with this condition presented with low-grade fevers and erythematous plaques, pustules, and bullae limited to the dorsal hands and fingers, which were characterized histologically by a dense neutrophilic infiltrate and leukocytoclastic vasculitis. We describe patients with a similar clinical presentation, but who lacked vasculitis on biopsy findings. We describe 3 otherwise asymptomatic patients with hemorrhagic bullae, plaques, and pustules solely on the dorsal hands. Biopsy specimens showed a neutrophilic infiltrate and leukocytoclasis, but no necrotizing vasculitis, and were reminiscent of Sweet's neutrophilic dermatoses. In our patients, corticosteroids or dapsone led to clearing of the lesions, and small maintenance doses of dapsone prevented their recurrence. Our 3 patients had clinical lesions similar to those termed pustular vasculitis of the hands, but which lacked leukocytoclastic vasculitis on biopsy findings. Because of histologic findings and a therapeutic response more characteristic of Sweet's syndrome, we propose the term neutrophilic dermatosis of the dorsal hands. In addition, low-dose dapsone is proposed as a possible first-line therapy in this condition, especially in those with recurrent disease.