Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria; School of Pathology, University of Milan, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy.
Dermopathology Unit, San Gallicano Dermatologic Institute, Rome, Italy.
J Am Acad Dermatol. 2018 Feb;78(2):377-382. doi: 10.1016/j.jaad.2017.08.041.
Hemophagocytosis is well known in cytotoxic cutaneous T-cell lymphomas (CTCLs), in which it may represent a sign of hemophagocytic lymphohistiocytosis syndrome (HLHS), and is also typical of cutaneous Rosai-Dorfman disease (cRDD) (without prognostic relevance). Only rarely, has cutaneous hemophagocytosis (CH) been described in other skin conditions.
To characterize the clinicopathologic features of CH in skin biopsy specimens from patients with conditions other than CTCL or cRDD.
Case series analyzing clinicopathologic features and follow-up data on patients presenting with histopathologic signs of CH.
Biopsy specimens from 21 patients were included. None of the patients had HLHS. The majority (n = 11) presented with leukocytoclastic vasculitis. Other associated diseases were lupus erythematous (n = 2), arthropod bite reaction (n = 2), erysipelas (n = 1), acne conglobata (n = 1), and Sweet syndrome (n = 1). Three patients had a nonspecific rash concomitant with Chlamydia pneumonia, middle ear infection, and pharyngitis, respectively.
This was a single-center, retrospective study.
Isolated CH in conditions other than CTCL and cRDD is a histopathologic finding related mostly to leukocytoclastic vasculitis. Extensive investigations should be performed only if patients have other signs or symptoms of HLHS.
噬血细胞现象在细胞毒性皮肤 T 细胞淋巴瘤（CTCL）中很常见，在这种情况下，它可能代表噬血细胞性淋巴组织细胞增生症（HLHS）的一个标志，也常见于皮肤 Rosai-Dorfman 病（cRDD）（无预后相关性）。只有极少数情况下，噬血细胞性皮肤病（CH）在其他皮肤疾病中被描述过。
描述除 CTCL 或 cRDD 以外的其他皮肤疾病患者皮肤活检标本中 CH 的临床病理特征。
对表现出 CH 组织病理学特征的患者的临床病理特征和随访数据进行病例系列分析。
共纳入 21 例患者的活检标本。这些患者均无 HLHS。大多数患者（n=11）表现为白细胞碎裂性血管炎。其他相关疾病包括红斑狼疮（n=2）、节肢动物叮咬反应（n=2）、丹毒（n=1）、聚合性痤疮（n=1）和Sweet 综合征（n=1）。3 例患者分别伴有肺炎衣原体、中耳感染和咽炎的非特异性皮疹。
这是一项单中心、回顾性研究。
除 CTCL 和 cRDD 以外的其他疾病中孤立性 CH 是一种组织病理学发现，主要与白细胞碎裂性血管炎有关。只有当患者有 HLHS 的其他体征或症状时，才应进行广泛的检查。
