Dermatology department, University Hospital of Paracelsus, Medical University of Salzburg, Salzburg, Austria.
Dermatol Ther. 2018 Jul;31(4):e12609. doi: 10.1111/dth.12609. Epub 2018 Apr 23.
Morbihan's disease is characterized by chronic persistent facial edema of the upper half of the face, absence of typical diagnostic findings, and refractoriness to treatment. A 44-year-old man was diagnosed with Morbihan's disease based on clinical signs and histopathology, which showed dermal edema in upper dermis, discrete lymphocytic infiltrate without granulomatous reaction, and mast cell infiltration. After long-term therapy with intralesional triamcinolone a remarkable objective and subjective clinical response was observed. Reported cases of Morbihan's disease are reviewed, with respect to their treatment and histopathological findings. Mast cell infiltration has been observed on histopathology in most patients who responded to intralesional triamcinolone, suggesting a possible marker of response. The long-lasting response seen in our case indicates the efficacy of intralesional triamcinolone in this rare condition.
莫比汉氏病的特征为慢性持续性面中部肿胀，无典型的诊断性发现，且对治疗有抗性。一名 44 岁男性，根据临床症状和组织病理学诊断为莫比汉氏病，其表现为真皮上部水肿，离散性淋巴细胞浸润，无肉芽肿反应，以及肥大细胞浸润。在接受曲安奈德皮损内注射治疗后，患者获得了显著的客观和主观临床缓解。本文回顾了报道的莫比汉氏病病例，探讨了其治疗和组织病理学发现。大多数对皮损内曲安奈德治疗有反应的患者，其组织病理学检查均可见肥大细胞浸润，这提示了可能的反应标志物。本病例的持久缓解表明了皮损内曲安奈德治疗这种罕见疾病的有效性。
