Hu Stephanie W, Robinson Maria, Meehan Shane A, Cohen David E
The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, USA.
Dermatol Online J. 2012 Dec 15;18(12):27.
Morbihan disease, which consists of solid facial edema, is a rare complication of rosacea, a common cutaneous disorder in middle-aged individuals. The characteristic features of Morbihan disease are its chronic course, typical clinical picture, lack of specific laboratory and histopathologic findings, and refractoriness to therapeutic measures. Since its initial description in 1957, only a small number of cases have been reported in the dermatologic literature. We report a 54-year-old man who developed a two-year duration of erythema and edema that affects the upper and mid face, with accentuation in the periorbital region. Patch tests excluded an allergic contact dermatitis and histopathologic investigation showed small, nodular clusters of epithelioid cells in the dermis that were consistent with sarcoidal granulomata. A diagnosis of Morbihan disease was made owing to the combination of clinical and histopathologic findings. Therapeutic options for the disease remain unsatisfactory and treatments reported in the literature include systemic glucocorticoids, oral tetracyclines, thalidomide, isotretinoin, ketotifen, and clofazimine. Our patient failed a six-to-seven months course of minocycline prior to presentation and has since experienced improvement on gradually-increasing doses of isotretinoin.
莫尔比昂病,表现为面部实性水肿,是酒渣鼻的一种罕见并发症,酒渣鼻是中年人群常见的皮肤疾病。莫尔比昂病的特征包括其慢性病程、典型临床表现、缺乏特异性实验室及组织病理学表现以及对治疗措施的难治性。自1957年首次描述以来,皮肤科文献中仅报道了少数病例。我们报告一名54岁男性,出现了持续两年的红斑和水肿,累及面部上半部分和中部,眶周区域更为明显。斑贴试验排除了过敏性接触性皮炎,组织病理学检查显示真皮内有小的结节状上皮样细胞簇,与结节病肉芽肿相符。综合临床和组织病理学表现,诊断为莫尔比昂病。该疾病的治疗选择仍不尽人意,文献报道的治疗方法包括全身用糖皮质激素、口服四环素、沙利度胺、异维A酸、酮替芬和氯法齐明。我们的患者在就诊前接受了六到七个月的米诺环素治疗但无效,此后逐渐增加异维A酸剂量后病情有所改善。