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进行性结节性组织细胞增生症:一种不常见的疾病。

Progressive nodular histiocytosis: an unusual disorder.

机构信息

Institute of Dermatology and Dermatologic Surgery (INDERMA), Guatemala.

出版信息

Dermatol Online J. 2021 Feb 15;27(2):13030/qt4t37r77d.

PMID:33818980
Abstract

Progressive nodular histiocytosis (PNH) is a rare type of non-Langerhans cell histiocytosis of the xanthogranuloma group. Less than 20 cases have been reported. We report here a novel case of PNH with dermoscopic description and post-surgical outcome. Our patient presented with pruritic papules and nodules with progression over two years. Dermoscopic examination of large lesions showed multiple telangiectases without ulceration. There was no mucosal involvement. Histopathological analysis of a papule showed a dermal infiltrate composed of histiocytes and many Touton giant cells; a nodule revealed a dermis occupied by spindle cells arranged in a storiform pattern. The nodules were resected and the largest surgical defect, on the forehead, was corrected with A-T advancement flap. The papules were treated with cryosurgery achieving adequate cosmetic outcome without recurrence.

摘要

进行性结节性组织细胞增生症(PNH)是一种罕见的黄色肉芽肿组中非朗格汉斯细胞组织细胞增生症,报道病例不足 20 例。我们在此报告一例 PNH 患者,描述了其临床皮损表现及术后转归。患者因瘙痒性丘疹和结节,进行性加重 2 年就诊。大皮损的皮肤镜检查显示多发性毛细血管扩张,无溃疡。无黏膜受累。丘疹的组织病理学分析显示真皮浸润由组织细胞和许多多核巨细胞组成;结节显示梭形细胞呈束状排列的真皮浸润。结节被切除,额部最大的手术缺损用 A-T 推进皮瓣矫正。丘疹采用冷冻疗法治疗,美容效果满意,无复发。

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