University Hospital Dr. Jose Eleuterio Gonzalez UANL, Anatomical Pathology and Cytopathology Department, Francisco I. Madero y Gonzalitos S/N, 64460 Monterrey, Nuevo Leon, Mexico.
Malays J Pathol. 2020 Apr;42(1):137-141.
Eosinophilic angiocentric fibrosis (EAF) is a rare lesion that predominantly affects the upper respiratory tract. Its etiology is unknown and it has been recently associated with the IgG4- related disease (IgG4-RD) spectrum. To the author's knowledge, this is the sixth case report of the relationship between EAF and IgG4-RD.
We report the case of a 37-year-old woman with nasal deformity and facial pain. The lesion was surgically excised. Histological examination revealed features of EAF with an IgG4/IgG plasma cell ratio ≷73% and 31 IgG4 stained cells per high power field. No clinical or radiological recurrence was detected during follow-up. Serum IgG4 quantification one year after surgery was within normal limits.
The relationship between both entities may have therapeutic impact because IgG4-RD of the head and neck has a high remission rate with corticosteroids and immunosuppressive therapy. Additional reports of this infrequent disease are necessary to elucidate appropriate treatment and prognosis.
嗜酸性血管中心性纤维化（EAF）是一种罕见的病变，主要影响上呼吸道。其病因不明，最近与 IgG4 相关疾病（IgG4-RD）谱相关。据作者所知，这是第六例 EAF 与 IgG4-RD 之间关系的病例报告。
我们报告了一例 37 岁女性，有鼻畸形和面部疼痛。病变被手术切除。组织学检查显示 EAF 的特征，IgG4/IgG 浆细胞比值≷73%，高倍视野中有 31 个 IgG4 染色细胞。在随访期间未发现临床或影像学复发。手术后一年的血清 IgG4 定量在正常范围内。
这两种疾病之间的关系可能具有治疗意义，因为头颈部的 IgG4-RD 用皮质类固醇和免疫抑制治疗有很高的缓解率。需要更多关于这种罕见疾病的报告来阐明适当的治疗和预后。
