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1例特发性肉芽肿性乳腺炎、化脓性汗腺炎和坏疽性脓皮病临床特征重叠病例经阿达木单抗成功治疗

A Case of Overlapping Clinical Features of Idiopathic Granulomatous Mastitis, Hidradenitis Suppurativa, and Pyoderma Gangrenosum Successfully Treated with Adalimumab.

作者信息

Breznik Vesna, Marko Pij Bogomir

机构信息

Department of Dermatovenerology, University Medical Centre Maribor, Maribor, Slovenia.

出版信息

Case Rep Dermatol. 2022 May 3;14(2):98-106. doi: 10.1159/000523801. eCollection 2022 May-Aug.

Abstract

A 36-year-old woman with a 6-month history of painful unilateral mammary nodules and abscesses was initially treated by gynecologists under the diagnosis of idiopathic granulomatous mastitis (IGM). IGM is an entity that has not been described in classical dermatological textbooks and is considered a rare inflammatory breast disease assumingly associated with trauma, infection, or autoimmune system manifestations. In this patient, the lesions were refractory to conventional treatment of IGM comprising of surgical incisions, systemic antibiotics, dexamethasone, and methotrexate. At the initial visit to the dermatology department, a working diagnosis of localized hidradenitis suppurativa (HS) of breast was established, and treatment with systemic doxycycline was initiated. After the diagnostic incisional biopsy, the inflamed nodule deteriorated into a painful ulceration, implying a pathergy phenomenon. Histopathological features were consistent with the granulomatous type of pyoderma gangrenosum (PG). Treatment with systemic methylprednisolone and mycophenolate mofetil was unsuccessful. Subsequently, the patient developed nodules in the inguinal and axillary areas, typical for HS. Finally, adalimumab treatment resulted in the complete resolution of all lesions without relapse even after the biologic therapy was discontinued. Although in this case, IGM was not confirmed histopathologically, we noted several etiopathological and therapeutic similarities between IGM, PG, and HS and summarized them in a unique table. Further observations are needed to ascertain the potential associations among the three entities.

摘要

一名36岁女性,有6个月单侧乳腺疼痛性结节及脓肿病史,最初由妇科医生诊断为特发性肉芽肿性乳腺炎(IGM)并进行治疗。IGM是一种在经典皮肤病学教科书中未被描述的疾病,被认为是一种罕见的炎症性乳腺疾病,推测与创伤、感染或自身免疫系统表现有关。在该患者中,病变对包括手术切开、全身用抗生素、地塞米松和甲氨蝶呤在内的IGM常规治疗无效。在初次就诊皮肤科时,确诊为乳腺局限性化脓性汗腺炎(HS),并开始使用全身性强力霉素治疗。诊断性切开活检后,发炎的结节恶化为疼痛性溃疡,提示有同形反应现象。组织病理学特征与坏疽性脓皮病(PG)的肉芽肿型一致。全身性甲泼尼龙和霉酚酸酯治疗无效。随后,患者在腹股沟和腋窝区域出现结节,这是HS的典型表现。最后,阿达木单抗治疗使所有病变完全消退,即使在生物治疗停药后也未复发。虽然在本例中,IGM在组织病理学上未得到证实,但我们注意到IGM、PG和HS之间在病因病理及治疗方面有若干相似之处,并将其总结在一张独特的表格中。需要进一步观察以确定这三种疾病之间的潜在关联。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa57/9149353/4b6db7fa48f5/cde-0014-0098-g01.jpg

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