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面部播散性粟粒性狼疮:1例对环孢素治疗有效的难治性病例。

Lupus miliaris disseminatus faciei: A resistant case with response to cyclosporine.

作者信息

Sardana Kabir, Chugh Shikha, Ranjan Rashmi, Khurana Nita

机构信息

Department of Dermatology, Ram Manohar Lohia Hospital, New Delhi, India.

Lok Nayak Hospital, Address, 107 Cruikshank Street, Frenchville, Rockhampton, QLD 4701, Australia.

出版信息

Dermatol Ther. 2017 Jul;30(4). doi: 10.1111/dth.12496. Epub 2017 Apr 26.

DOI:10.1111/dth.12496
PMID:28447377
Abstract

Lupus miliaris disseminatus faciei (LMDF) is a chronic, inflammatory dermatosis of unknown etiology, characterized by multiple, monomorphic, symmetrical, reddish-brown papules over forehead, cheeks, and eyelids. Histopathology shows perifollicular epitheloid cell granuloma. Though numerous therapies, ranging from cyclines, macrolides, dapsone tranilast, isotretinoin, steroids, and tacrolimus have been tried, the results are inconsistent, except with systemic steroids. One approach is to administer therapies based on the histological findings and the corresponding mode of action of drugs, thus antibiotics and dapsone are effective in the early inflammatory stage while clofazamine can be used in the granulomatous stage of the disease. A case of LMDF, recalcitrant to multiple systemic therapies, who responded dramatically to cyclosporine (50 mg twice daily), which probably was due to the specific effect on T 1 cell response which mediates cell mediated immunity responsible for granulomatous changes on histology has been reported. This case highlights that LMDF is an independent granulomatous entity (not a variant of rosacea or tuberculosis).

摘要

面部播散性粟粒性狼疮(LMDF)是一种病因不明的慢性炎症性皮肤病,其特征为在前额、脸颊和眼睑出现多个单形性、对称性、红棕色丘疹。组织病理学表现为毛囊周围上皮样细胞肉芽肿。尽管已经尝试了多种治疗方法,包括四环素类、大环内酯类、氨苯砜、曲尼司特、异维A酸、类固醇和他克莫司,但除全身用类固醇外,结果并不一致。一种方法是根据组织学发现和药物相应的作用方式进行治疗,因此抗生素和氨苯砜在早期炎症阶段有效,而氯法齐明可用于疾病的肉芽肿阶段。有报道称,1例对多种全身治疗均耐药的LMDF患者对环孢素(每日2次,每次50mg)反应显著,这可能是由于环孢素对T1细胞反应有特异性作用,而T1细胞反应介导了细胞介导免疫,这种免疫在组织学上导致了肉芽肿性改变。该病例突出表明,LMDF是一种独立的肉芽肿性疾病(不是酒渣鼻或结核的变种)。

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引用本文的文献

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Recalcitrant lupus miliaris disseminatus faciei improved by cyclosporine monotherapy.环孢素单一疗法改善了顽固性面部播散性粟粒性狼疮。
JAAD Case Rep. 2022 Jul 6;27:16-19. doi: 10.1016/j.jdcr.2022.06.038. eCollection 2022 Sep.
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A case of lupus miliaris disseminatus faciei after allogeneic hematopoietic stem cell transplantation.异基因造血干细胞移植后发生的面部播散性粟粒性狼疮1例。
Chin Med J (Engl). 2019 Sep 5;132(17):2133-2134. doi: 10.1097/CM9.0000000000000411.