難治性眼瞼浮腫を主訴としMorbihan病と診断した2例

Two Cases of Morbihan Disease with Refractory Eyelid Edema

はじめに

　Morbihan病は顔面に慢性的な腫脹を来たすまれな疾患である 1）。Morbihan病の病態はいまだに明らかではないが，酒皶などを起因として顔面の皮膚・皮下のリンパうっ滞を来たし，リンパ浮腫が生じている可能性が指摘されている 2）。疾患特異的なマーカーは存在せず，病歴および血液検査や病理組織学的検査の所見を総合し，顔面の腫脹を来たすそのほかの鑑別疾患を除外してMorbihan病と診断されている。Morbihan病の多くは治療抵抗性であり，内服治療や外科的な治療法に関する症例報告は複数あるものの，現在までに標準治療が確立されるまでには至っていない 3）。

　今回われわれは，難治性眼瞼腫脹と視野障害を主訴としたMorbihan病の2例を経験した。眼瞼の疾患を扱う形成外科医からも見逃され，放置されている症例が多いと思われ，Morbihan病と診断し治療に至るまでの経過および今後の治療法の展望について，文献的考察を踏まえて報告する。

　Morbihan disease is a rare condition characterized by persistent edema and erythema of the upper half of the face. Although the etiology of the disease is unknown, a histopathological examination reveals dermal edema and inflammatory cell infiltration around the blood vessels and lymph vessels. Facial lymphedema is typically severe in the periocular region, causing visual field defects. Morbihan disease can be diagnosed only when all other illnesses with similar clinical symptoms have been ruled out. There are a few treatments for Morbihan disease, e.g., systemic corticosteroids, antibiotics, and blepharoplasty, but they provide only temporary or partial relief.

　We describe the cases of two patients with Morbihan disease, both of whom presented with persistent eyelid swelling. The first patient was a 25-year-old woman who had experienced left upper-eyelid edema for 1 year. Although she was administered several oral medications, the edema did not respond and she developed vision impairment. She was thus referred to our department. We performed a left blepharoplasty. At 1 year and 6 months later, a right blepharoplasty was performed to correct the width of the right eyelid fold. After these surgical treatments the patientʼs visual field improved, and the edema has not recurred. The second patient was a 71-year-old man who had suffered from bilateral eyelid edema for 1 year. He was referred to our department because of entropion of both lower eyelids, and a lower-eyelid blepharoplasty was performed. However, edema recurred in both upper eyelids after the surgery, and an upper-eyelid blepharoplasty was performed. Based on the patientʼs symptoms and laboratory results, he was subsequently diagnosed with Morbihan disease. Although he was administered minocycline 200 mg/day, his symptoms persisted.