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坏死性黄色肉芽肿——病例报告及文献综述

Necrobiotic Xanthogranuloma - Case Report and Literature Review.

作者信息

Geoloaica Liliana Gabriela, Pătraşcu Virgil, Ciurea Raluca Niculina

机构信息

Department of Dermatology, Emergency County Hospital, Craiova, University of Medicine and Pharmacy of Craiova, Romania.

Department of Pathology, Emergency County Hospital, Craiova, University of Medicine and Pharmacy of Craiova, Romania.

出版信息

Curr Health Sci J. 2021 Jan-Mar;47(1):126-131. doi: 10.12865/CHSJ.47.01.21. Epub 2021 Mar 31.

Abstract

Necrobiotic xanthogranuloma is a rare type of non-Langerhans histiocytosis, whose main clinical features are the development of red-brown, purplish or yellowish skin papules and nodules, which evolve by forming infiltrated plaques. The periorbital region is the most commonly affected site. Some cases have lesions on the torso or extremities, with no facial involvement. Extracutaneous involvement of the ocular, respiratory, and cardiac tissues have also been described. Most patients have an associated monoclonal gammopathy (IgG k and λ). The treatment is difficult, with progression and recurrence. We present the clinical case of a 65-year-old woman, who was hospitalized for multiple erythematous plaques and placards, with fine squames and telangiectasis on the surface, disseminated within the scalp, ears, trunk, lower limbs; some plaques have a circinate border with reddish-purple, slightly protruding edges and a whitish and erosive atrophic center. The lesions within the scalp are alopecic. The disease began 15 years ago, the patient being diagnosed with Psoriasis vulgaris and treated with dermatocorticoids and Cignolin, with no remarkable results. Paraclinical investigations did not reveal any associated pathologies. Histopathological and immunohistochemical examination confirmed the diagnosis of necrobiotic Xanthogranuloma. The patient was treated with antihistamines, Neuromultivit, Vit E 100mg/day, Oximed spray, Atoderm emollient cream, Neopreol ointment, with slow favorable evolution. The physical examination and laboratory investigations for the diagnosis and surveillance of malignant diseases should be performed on a regular basis in patients with NXG. Our patient had lesions with a course of 15 years, with no development of multiple myeloma or other systemic involvement.

摘要

坏死性黄色肉芽肿是一种罕见的非朗格汉斯组织细胞增多症,其主要临床特征是出现红棕色、紫色或淡黄色皮肤丘疹和结节,这些病变会逐渐形成浸润性斑块。眶周区域是最常受累的部位。有些病例在躯干或四肢有病变,面部未受累。也有眼部、呼吸道和心脏组织的皮肤外受累的描述。大多数患者伴有单克隆丙种球蛋白病(IgG κ和λ)。治疗困难,病情会进展和复发。我们报告一例65岁女性的临床病例,该患者因头皮、耳部、躯干、下肢散在分布的多个红斑性斑块和斑片入院,表面有细小鳞屑和毛细血管扩张;一些斑块有环状边界,边缘呈红紫色,略突出,中心为白色且糜烂萎缩。头皮内的病变导致脱发。该病始于15年前,患者曾被诊断为寻常型银屑病,接受过皮质类固醇和西诺林治疗,但效果不佳。辅助检查未发现任何相关病变。组织病理学和免疫组织化学检查确诊为坏死性黄色肉芽肿。患者接受了抗组胺药、神经复合维生素、每日100毫克维生素E、奥昔美定喷雾剂、阿托德润肤霜、新泼罗软膏治疗,病情缓慢好转。对于坏死性黄色肉芽肿患者,应定期进行体格检查和实验室检查以诊断和监测恶性疾病。我们的患者病变病程长达15年,未发生多发性骨髓瘤或其他全身受累情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/13d1/8200611/26bc56fd2bd9/CHSJ-47-01-126-fig9.jpg

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