Innocenti Alessandro, Innocenti Marco, Taverna Cecilia, Melita Dario, Mori Francesco, Ciancio Francesco, De Giorgi Vincenzo, Parronchi Paola, Vultaggio Alessandra, Matucci Andrea
Plastic and Reconstructive Microsurgery, Department of Surgery and Translational Medicine, Careggi University Hospital, Florence, Italy.
Plastic and Reconstructive Microsurgery, Department of Surgery and Translational Medicine, Careggi University Hospital, Florence, Italy.
Int J Surg Case Rep. 2017;31:241-244. doi: 10.1016/j.ijscr.2017.01.062. Epub 2017 Jan 30.
Miescher's cheilitis is clinically characterized by persistent swelling of the lip(s). Its pathogenesis is still unknown. Histopathologically is characterized by sub-epithelial edema, increased number of dilated lymphatic vessels and an inflammatory infiltrate and/or non-caseating/non- necrotic granulomas. Even if the disorder must be controlled by medical therapy, surgery may be required to treat most severe cases.
We report a 30-year-old man who presented a persistent swelling of both lips since 8 years, previously treated with intralesional steroid and immunosuppressive therapy. Clinical examination did not show facial nerve palsy or other associated conditions. On the base of clinical and histopathological findings, a diagnosis of Miescher's syndrome was made. Patient underwent Conway's reduction cheilopasty repaired with local flaps. At one-year follow-up, the patient does not show local recurrence of the deformity; both oral continence and lip sensation are preserved.
Because of its extreme rarity and unknown etiopathogenesis, Miescher's cheilitis receives poor attention and may often remain misdiagnosed. Several medical therapies are proposed, in particular steroids and immunosuppression. Even if medical therapy remains the main treatment, surgery may be required.
Satisfactory results have been obtained combining medical therapy and surgical approach.
米舍尔唇炎的临床特征为唇部持续肿胀。其发病机制尚不清楚。组织病理学特征为上皮下水肿、扩张的淋巴管数量增加以及炎症浸润和/或非干酪样/非坏死性肉芽肿。即使该病症必须通过药物治疗来控制，但对于大多数严重病例可能仍需要手术治疗。
我们报告一名30岁男性，自8年前起双唇持续肿胀，此前接受过病损内类固醇和免疫抑制治疗。临床检查未发现面神经麻痹或其他相关病症。根据临床和组织病理学检查结果，诊断为米舍尔综合征。患者接受了用局部皮瓣修复的康威缩唇成形术。在一年的随访中，患者未出现畸形的局部复发；口腔节制功能和唇部感觉均得以保留。
由于米舍尔唇炎极为罕见且病因不明，因此受到的关注较少，常常可能被误诊。人们提出了多种药物治疗方法，尤其是类固醇和免疫抑制治疗。即使药物治疗仍然是主要治疗方法，但可能仍需要手术治疗。
药物治疗和手术方法相结合取得了令人满意的效果。
