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嗜酸粒细胞性筋膜炎和深部硬皮病的临床病理和免疫表型特征:27 例对比研究。

Clinicopathologic and immunophenotypic features of eosinophilic fasciitis and morphea profunda: A comparative study of 27 cases.

机构信息

Department of Dermatology, Mayo School of Graduate Medical Education, Mayo Clinic, Rochester, Minnesota.

Department of Dermatology, Mayo School of Graduate Medical Education, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo School of Graduate Medical Education, Mayo Clinic, Rochester, Minnesota.

出版信息

J Am Acad Dermatol. 2018 Jan;78(1):121-128. doi: 10.1016/j.jaad.2017.06.148. Epub 2017 Aug 31.

DOI:10.1016/j.jaad.2017.06.148
PMID:28865864
Abstract

BACKGROUND

Eosinophilic fasciitis (EF) and morphea profunda (MP) are inflammatory and sclerosing disorders of the subcutis that can exhibit clinical and pathologic presentations that overlap.

OBJECTIVE

To identify clinicopathologic features that can be used to distinguish EF from MP.

METHODS

We performed a retrospective review of 16 patients with EF and 11 patients with MP. Hematoxylin-eosin, CD123, CD34, and Verhoeff-Van Gieson stains were evaluated on skin biopsies that included the fascia.

RESULTS

EF patients were more likely than MP patients to be men (P = .047), have forearm involvement (P = .003), and have peripheral eosinophilia (P < .01). Compared with MP patients, patients with EF were more likely to have fascia that contained eosinophils (P = .003), although eosinophils were absent in 3 (19%) patients with EF. Focal absence of CD34 staining was more prominent in the fascia of EF patients (P = .04). The extent of Verhoeff-Van Gieson staining did not differ between the 2 groups. Dermal sclerosis was not detected in many cases of EF and MP (56% and 36%, respectively).

LIMITATIONS

This was a retrospective study at a single institution.

CONCLUSION

Although EF and MP share clinical and pathologic features, our results indicate that the presence of eosinophils in the blood and fascia and focal loss of CD34 staining might be more suggestive of EF than MP.

摘要

背景

嗜酸性筋膜炎(EF)和深部硬斑病(MP)是皮下组织的炎症性和硬化性疾病,其临床表现和病理表现可能重叠。

目的

确定可用于区分 EF 和 MP 的临床病理特征。

方法

我们对 16 例 EF 患者和 11 例 MP 患者进行了回顾性研究。对包括筋膜在内的皮肤活检进行苏木精-伊红、CD123、CD34 和 Verhoeff-Van Gieson 染色。

结果

EF 患者比 MP 患者更可能是男性(P =.047),前臂受累(P =.003),外周血嗜酸性粒细胞增多(P <.01)。与 MP 患者相比,EF 患者的筋膜中更可能含有嗜酸性粒细胞(P =.003),尽管 EF 中有 3 例(19%)患者中没有嗜酸性粒细胞。EF 患者的筋膜中 CD34 染色的局灶性缺失更为明显(P =.04)。两组之间的 Verhoeff-Van Gieson 染色程度没有差异。EF 和 MP 的许多病例均未检测到真皮硬化(分别为 56%和 36%)。

局限性

这是一项单中心的回顾性研究。

结论

尽管 EF 和 MP 具有共同的临床和病理特征,但我们的结果表明,血液和筋膜中嗜酸性粒细胞的存在以及 CD34 染色的局灶性缺失可能更提示 EF 而非 MP。

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