Broekaert Sigrid M C, Böer-Auer Almut, Kerl Katrin, Herrgott Ilka, Schulz Xenia, Bonsmann Gisela, Brehler Randolf, Metze Dieter
*Department of Dermatology, University of Münster, Münster, Germany; †Dermatopathology Laboratory, Dermatologikum, Hamburg, Germany; ‡Department of Dermatology, University of Zürich, Zürich, Switzerland; and §Department of Medical Statistics, University Medical Center Göttingen, Göttingen, Germany.
Am J Dermatopathol. 2016 Jan;38(1):39-49. doi: 10.1097/DAD.0000000000000390.
Neutrophilic urticarial dermatosis (NUD) comprises a particular autoinflammatory condition within the spectrum of aseptic neutrophilic dermatoses characterized by a distinct urticarial eruption clinically and a neutrophilic dermatosis histopathologically.
In this study, we reviewed skin biopsies of lesional skin of patients seen in our outpatient clinic for autoimmune dermatoses and in allergy department from 1982 to 2014 that fulfilled these criteria.
A total of 77 biopsies from 50 patients were analyzed histopathologically. Included were cases of Schnitzler syndrome, Still disease, systemic lupus erythematosus, Sjögren syndrome, cryopyrin-associated periodic syndrome, primary biliary cirrhosis, inflammatory bowel disease, and those that had signs of systemic inflammation not otherwise specified, that is, fever, arthritis, leukocytosis, and elevated erythrocyte sedimentation rate. A control cohort was defined as including a total of 70 biopsies from 50 patients comprising neutrophilic urticaria (pressure-induced and not pressure-induced), conventional urticaria, lupus erythematosus expressing neutrophils, and exanthematous drug reaction of macular type expressing neutrophils.
Skin biopsies of NUD revealed a perivascular and interstitial neutrophilic infiltrate focally extending into the epithelia of epidermis, hair follicles, sebaceous and sweat glands, a feature which we termed neutrophilic epitheliotropism. This neutrophilic epitheliotropism proved to be of high sensitivity (83.1%) and lower specificity (74.3%). The histological findings could be substantiated by immunohistochemical markers for leukocytes (elastase and myeloperoxidase), in particular in cases where neutrophils showed uncharacteristic band-like nuclei.
Neutrophilic epitheliotropism is a new sensitive and specific histopathological clue for NUD, a histopathological reaction pattern within the spectrum of neutrophilic dermatoses that needs to be differentiated from conventional urticaria.
嗜中性粒细胞性荨麻疹性皮肤病（NUD）是无菌性嗜中性粒细胞性皮肤病谱中的一种特殊的自身炎症性疾病，其临床特征为独特的荨麻疹样皮疹，组织病理学表现为嗜中性粒细胞性皮肤病。
在本研究中，我们回顾了1982年至2014年在我们门诊的自身免疫性皮肤病诊所和过敏科就诊的符合这些标准的患者皮损的皮肤活检标本。
对50例患者的77份活检标本进行了组织病理学分析。包括施尼茨勒综合征、成人斯蒂尔病、系统性红斑狼疮、干燥综合征、冷吡啉相关周期性综合征、原发性胆汁性肝硬化、炎症性肠病，以及那些有未明确说明的全身炎症体征的病例，即发热、关节炎、白细胞增多和红细胞沉降率升高。定义一个对照组，包括50例患者的70份活检标本，其中有嗜中性粒细胞性荨麻疹（压力性和非压力性）、传统荨麻疹、表达嗜中性粒细胞的红斑狼疮，以及表达嗜中性粒细胞的斑疹型药疹。
NUD的皮肤活检显示血管周围和间质有嗜中性粒细胞浸润，局部延伸至表皮、毛囊、皮脂腺和汗腺上皮，我们将这一特征称为嗜中性粒细胞亲上皮性。这种嗜中性粒细胞亲上皮性具有较高的敏感性（83.1%）和较低的特异性（74.3%）。白细胞免疫组化标记物（弹性蛋白酶和髓过氧化物酶）可证实组织学 findings，特别是在嗜中性粒细胞显示非典型带状核的病例中。
嗜中性粒细胞亲上皮性是NUD的一种新的敏感且特异的组织病理学线索，是嗜中性粒细胞性皮肤病谱中的一种组织病理学反应模式，需要与传统荨麻疹相鉴别。
