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[深部肉芽肿性坏疽性脓皮病:一种新的变异型?]

[Deep-seated granulomatous pyoderma gangrenosum: A novel variant?].

作者信息

Baline K, Khadir K, Chiheb S, Marnissi F, Benchikhi H

机构信息

Service de dermatologie et de vénéréologie, CHU Ibn Rochd, rue Lahcen Laarjoune, 20000 Casablanca, Maroc.

Service de dermatologie et de vénéréologie, CHU Ibn Rochd, rue Lahcen Laarjoune, 20000 Casablanca, Maroc.

出版信息

Ann Dermatol Venereol. 2015 May;142(5):340-5. doi: 10.1016/j.annder.2014.11.010. Epub 2015 Jan 27.

DOI:10.1016/j.annder.2014.11.010
PMID:25637110
Abstract

BACKGROUND

We report an extremely rare case of pyoderma gangrenosum with the clinical features of pyoderma gangrenosum but with unusual histopathological findings and deep massive granulomatous infiltration through the dermis.

PATIENTS AND METHODS

A 15-year-old girl presented with a two-month history of deep ulcers on the lower legs and forehead. She also presented abdominal pain with diarrhea and vomiting that disappeared after symptomatic treatment. Histology showed extensive granulomatous infiltration in the dermis and hypodermis and a micro-abscess in the dermis. An abdominopelvic CT scan revealed peritoneal fluid and tissue formation in a lateral carotid cave (6.56 cm/4.56 cm). Colonoscopy was normal. The patient was treated with oral prednisone, resulting in resolution of the problems of cutaneous lesions, peritoneal fluid and tissue formation.

DISCUSSION

Our case involves a rare presentation of pyoderma gangrenosum (PG) inconsistent with any of the four clinical variants of PG and characterized histologically by the presence of deep granulomas in the dermis and hypodermis. Despite the atypical clinical presentation and unusual histopathological findings, PG seemed the most likely diagnosis after infectious diseases had been ruled out; the good outcome achieved with oral corticosteroids supported our diagnosis.

摘要

背景

我们报告了一例极为罕见的坏疽性脓皮病病例,其具有坏疽性脓皮病的临床特征,但组织病理学表现异常,真皮层有深部大量肉芽肿浸润。

患者与方法

一名15岁女孩,小腿和前额出现深部溃疡达两个月。她还伴有腹痛、腹泻和呕吐,经对症治疗后消失。组织学检查显示真皮层和皮下组织有广泛的肉芽肿浸润,真皮层有微脓肿。腹部盆腔CT扫描显示颈动脉外侧隐窝有腹腔积液和组织形成(6.56厘米/4.56厘米)。结肠镜检查正常。患者接受口服泼尼松治疗后,皮肤病变、腹腔积液和组织形成问题均得到解决。

讨论

我们的病例是坏疽性脓皮病(PG)的一种罕见表现,与PG的四种临床变型均不相符,组织学特征为真皮层和皮下组织存在深部肉芽肿。尽管临床表现不典型且组织病理学表现异常,但在排除感染性疾病后,PG似乎是最可能的诊断;口服糖皮质激素取得的良好疗效支持了我们的诊断。

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