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嗜酸性坏死性毛囊炎作为特应性素质的一种表现。

Necrotizing eosinophilic folliculitis as a manifestation of the atopic diathesis.

作者信息

Magro C M, Crowson A N

机构信息

Division of Dermatology, Department of Pathology, Ohio State University, Columbus, Ohio, USA.

出版信息

Int J Dermatol. 2000 Sep;39(9):672-7. doi: 10.1046/j.1365-4362.2000.00973.x.

DOI:10.1046/j.1365-4362.2000.00973.x
PMID:11044192
Abstract

BACKGROUND

Eosinophilic folliculitis (EF) is an idiopathic eruption of sterile pustules and papules involving the trunk, face, and extremities, associated in many cases with human immunodeficiency virus (HIV) infection. The classic histopathology is one of follicular-based, eosinophilic spongiosis with variable microabscess formation. We describe nine HIV-negative patients who manifested a novel form of pustular EF in the setting of atopy.

MATERIALS AND METHODS

Paraffin sections of skin biopsies from ten patients, stained with hematoxylin and eosin and special preparations to evaluate for microbial pathogens, were examined. Detailed clinical histories and serologic studies were obtained.

RESULTS

Among the clinical presentations in seven men, two women, and one girl (age range, 11-62 years) were ulcerative and/or nodular plaques mainly on the face and/or extremities, sometimes in an annular configuration. The clinical considerations included deep mycotic infection, ulcerative herpes, systemic vasculitis, Mucha Haberman disease, and pyoderma gangrenosum. All patients had a personal and/or family history of atopy. Co-existent medical illnesses included psoriasis, lupus erythematosus, and lymphoproliferative disease. One patient was on a calcium channel blocker, one on multiple antidepressants, and two on antihistamines, all of which are associated with immune dysregulation. All skin biopsies showed variable intra- follicular eosinophilic microabscesses, follicular necrosis, folliculocentric necrotizing eosinophilic vasculitis, marked degeneration of connective tissue fiber elements, and striking tissue eosinophilia, including flame figure formation and dermal eosinophilic abscesses. Apart from commensals, such as Pityrosporum and Demodex, microbial pathogens were not identified.

CONCLUSIONS

The presentations differed from conventional EF by virtue of a strong association with atopy and by the presence of ulceration, nodule formation, follicular and dermal necrosis, and eosinophilic vasculitis. We propose the term "necrotizing eosinophilic folliculitis," and suggest that the basis of this novel form of EF is an unrepressed T-helper lymphocyte type 2 (Th2)-dominant response to various epicutaneous stimuli in patients with atopy, the prototypic immune dysregulatory state associated with a Th2-dominant cytokine milieu.

摘要

背景

嗜酸性粒细胞性毛囊炎(EF)是一种特发性无菌性脓疱和丘疹性皮疹,累及躯干、面部和四肢,在许多情况下与人类免疫缺陷病毒(HIV)感染相关。经典的组织病理学表现为以毛囊为基础的嗜酸性海绵形成,并伴有不同程度的微脓肿形成。我们描述了9例在特应性背景下表现为一种新型脓疱性EF的HIV阴性患者。

材料与方法

对10例患者皮肤活检的石蜡切片进行苏木精-伊红染色及特殊制片以评估微生物病原体,并进行检查。获取详细的临床病史和血清学研究结果。

结果

7名男性、2名女性和1名女孩(年龄范围11 - 62岁)的临床表现为主要位于面部和/或四肢的溃疡性和/或结节性斑块,有时呈环状。临床考虑包括深部真菌感染、溃疡性疱疹、系统性血管炎、穆恰 - 哈伯曼病和坏疽性脓皮病。所有患者均有个人和/或家族特应性病史。并存的内科疾病包括银屑病、红斑狼疮和淋巴增殖性疾病。1例患者正在服用钙通道阻滞剂,1例服用多种抗抑郁药,2例服用抗组胺药,所有这些都与免疫失调有关。所有皮肤活检均显示不同程度的毛囊内嗜酸性微脓肿、毛囊坏死、以毛囊为中心的坏死性嗜酸性血管炎、结缔组织纤维成分的显著变性以及明显的组织嗜酸性粒细胞增多,包括火焰状图形形成和真皮嗜酸性脓肿。除了如马拉色菌和蠕形螨等共生菌外,未发现微生物病原体。

结论

这些表现与传统的EF不同,因为它与特应性密切相关,且存在溃疡、结节形成、毛囊和真皮坏死以及嗜酸性血管炎。我们提出“坏死性嗜酸性粒细胞性毛囊炎”这一术语,并认为这种新型EF的基础是特应性患者(与以Th2为主导的细胞因子环境相关的典型免疫失调状态)对各种表皮刺激的未受抑制的2型辅助性T淋巴细胞(Th2)主导反应。

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