The Spectrum of Histopathologic Findings in Cutaneous Lesions in Patients With Still Disease

The Spectrum of Histopathologic Findings in Cutaneous Lesions in Patients With Still Disease
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DOI:
10.1309/ajcpze77uapsmdcd
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发表时间:
2015-12-01
影响因子:
3.5
通讯作者:
Granter, Scott R.
Granter, Scott R.
中科院分区:
医学4区
文献类型:
--
作者:
Larson, Allison R.;Laga, Alvaro C.;Granter, Scott R.

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目的:斯蒂尔病是一种罕见的疾病,其特点是血清阴性关节痛/关节炎,尖峰热,无论是一个短暂的鲑鱼色皮疹或持续性丘疹和plaques.Methods:我们描述了10例患者的临床和活检结果与短暂的皮疹斯蒂尔diseases.Results:14例活检标本进行了研究,从7名女性和3名男性,平均年龄为44.4岁。皮肤病变通常为红斑、丘疹或斑块,中位持续时间为5周。所有患者均有全身症状,包括发热和关节痛。在6份活检标本中,浸润主要为淋巴细胞性,在4份活检标本中,浸润主要为淋巴细胞性和嗜中性,在4份活检标本中,浸润主要为(但并非完全)嗜中性。其他研究结果包括局灶性空泡界面的变化,嗜中性小汗腺炎,表皮中性粒细胞,真皮粘蛋白,棘皮症与许多上表皮角化不良cells.Conclusions:重要的是要知道广泛的组织学谱,可能会遇到斯蒂尔病,并考虑斯蒂尔病的嗜中性粒细胞丰富,淋巴细胞丰富,混合性炎症性皮肤病的鉴别诊断。虽然在一过性皮疹的活检标本中观察到的组织学结果是非特异性的,但也存在一种独特的变体,其特征在于显著的表皮细胞凋亡,尤其是涉及上层。
Objectives: Still disease is a rare disorder characterized by seronegative arthralgias/arthritis, spiking fever, and either an evanescent salmon-colored rash or persistent papules and plaques.Methods: We describe the clinical and biopsy findings in 10 patients with the evanescent rash of Still disease.Results: Fourteen biopsy specimens were studied from seven women and three men with a mean age of 44.4 years. The skin lesions were typically erythematous macules, papules, or plaques with a median duration of 5 weeks. All patients had systemic symptoms, including fever and arthralgias. The infiltrate was predominantly lymphocytic in six biopsy specimens, approximately equal lymphocytic and neutrophilic in four biopsy specimens, and predominantly (although never exclusively) neutrophilic in four biopsy specimens. Other findings included focal vacuolar interface changes, neutrophilic eccrine hidradenitis, epidermal neutrophils, dermal mucin, and acanthosis associated with numerous upper epidermal dyskeratotic cells.Conclusions: It is important to be aware of the broad histologic spectrum that may be encountered in Still disease and to consider Still disease in the differential diagnosis of neutrophil-rich, lymphocyte-rich, and mixed inflammatory dermatoses. While the histologic findings seen in biopsy specimens of the evanescent rash are nonspecific, a distinctive variant also exists characterized by prominent epidermal apoptosis, especially involving the upper layers.