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1例角层下脓疱性皮肤病与坏疽性脓皮病并存病例及文献复习

A Case of the Co-Existence of Subcorneal Pustular Dermatosis and Pyoderma Gangrenosum and a Review of the Literature.

作者信息

Ferrillo Maria, Villani Alessia, Fabbrocini Gabriella, Mascolo Massimo, Megna Matteo, Costa Claudia, Napolitano Maddalena

机构信息

Section of Dermatology, Department of Clinical Medicine and Surgery, Dermatology Unit, University of Naples Federico II, Napoli, Italy.

Department of Advanced Biomedical Sciences, Pathology Section University of Naples Federico II, Napoli, Italy.

出版信息

Open Access Maced J Med Sci. 2018 Jul 8;6(7):1271-1274. doi: 10.3889/oamjms.2018.214. eCollection 2018 Jul 20.

Abstract

BACKGROUND

Subcorneal pustular dermatosis, also known as Sneddon-Wilkinson disease, can be classified as one of the neutrophilic dermatoses together with pyoderma gangrenosum. The development of both SPD and PG in the same patient has rarely been reported and may be a strong indicator of IgA dysglobulinemia.

CASE REPORT

We report the case of a 34-year-old woman with a 2-year history of relapsing pustular eruptions mainly affecting the abdomen, gluteus region, elbows, and the extremities. Four years after the onset of subcorneal pustular dermatosis (SPD), she developed pyoderma gangrenosum (PG) on her right hand. In literature, the coexistence of SPD and PG in the same patient has already been described. This co-occurrence might indicate a certain predisposition for immune dysregulation.

CONCLUSION

Although the two NDs are often associated with systemic diseases, these patients should be followed up for any malignancy because of the strong association between these disorders.

摘要

背景

角层下脓疱性皮肤病,也称为斯内登 - 威尔金森病,可与坏疽性脓皮病一同归类为嗜中性皮肤病。同一患者同时发生角层下脓疱性皮肤病(SPD)和坏疽性脓皮病(PG)的情况鲜有报道,这可能是IgA球蛋白异常血症的一个有力指标。

病例报告

我们报告一例34岁女性患者,有复发性脓疱疹病史2年,主要累及腹部、臀部、肘部和四肢。在角层下脓疱性皮肤病(SPD)发病4年后,她右手出现了坏疽性脓皮病(PG)。文献中已经描述过同一患者中SPD和PG并存的情况。这种共存可能表明存在某种免疫调节异常的易感性。

结论

虽然这两种嗜中性皮肤病常与全身性疾病相关,但由于这些疾病之间存在密切关联,应对这些患者进行随访以排查任何恶性肿瘤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6575/6062289/d2abc9c8269e/OAMJMS-6-1271-g001.jpg

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