Spallek G, Buttgereit F, Audring H, Hiepe F
Medizinische Universitätsklinik und Poliklinik III, Universitätsklinikum Charité, Humboldt-Universität zu Berlin.
Hautarzt. 1997 Nov;48(11):828-33. doi: 10.1007/s001050050669.
A 54-year-old, obese woman suffered from massive symmetrical swelling of the face, especially of the upper and lower eyelids. Initially the swelling occurred intermittently, but after 2 years it was permanent and progressive markedly limiting her visual fields. Neither laboratory findings nor imaging procedures provided any firm evidence of an underlying cardiac, renal or endocrinological disease. There was no suggestion of a storage disease. Skin biopsy showed foam cells and granulomatous inflammation, so the patient was tentatively diagnosed as having a monosymptomatic Melkersson-Rosenthal Syndrome. Eyelid surgery was performed to improve her visual fields. Treatment with clofazimine 100 mg daily was initiated. Regular follow-up visits over 7 months revealed no evidence of recurrence. The patient died a sudden cardiac death a few months later. The relatives refused an autopsy. The definite cause of her facial swelling remains unclear as we discuss the differential diagnostic possibilities.
一名54岁的肥胖女性面部出现大量对称性肿胀，尤其是上下眼睑。起初肿胀呈间歇性发作，但2年后肿胀持续存在且逐渐加重，严重限制了她的视野。实验室检查结果和影像学检查均未提供任何确凿证据表明存在潜在的心脏、肾脏或内分泌疾病。也没有提示存在贮积病。皮肤活检显示有泡沫细胞和肉芽肿性炎症，因此该患者初步诊断为单症状性梅克尔森 - 罗森塔尔综合征。进行了眼睑手术以改善她的视野。开始每日服用100毫克氯法齐明进行治疗。7个月的定期随访未发现复发迹象。几个月后，患者突发心源性死亡。亲属拒绝进行尸检。在我们讨论鉴别诊断可能性时，她面部肿胀的确切原因仍不清楚。
