Zhou Li-Feng, Lu Rong
Department of Dermatology, The 942 Hospital of the PLA Joint Logistic Support Force, Yinchuan 750004, Ningxia Hui Autonomous Region, China.
Department of Pathology, The 942 Hospital of the PLA Joint Logistic Support Force, Yinchuan 750004, Ningxia Hui Autonomous Region, China.
World J Clin Cases. 2022 Jul 6;10(19):6688-6694. doi: 10.12998/wjcc.v10.i19.6688.
Morbihan disease is a rare cutaneous disorder characterized by non-pitting edema and erythema of the upper two-thirds of the face. In severe cases, orbital and facial contour changes may affect the visual field, and there is no guideline for the standard treatment of this disease. Existing treatment methods have been reported to be associated with long medication cycle, easy recurrence after drug withdrawal, and multiple adverse reactions.
A 55-year-old Chinese woman presented to our hospital with non-pitting edema and erythema of the upper two thirds of her face for 5 mo. Physical examination showed obvious edema and erythema on the upper face. The boundary was unclear, the lesions were hard and non-pitting, and infiltration was obvious by touch. Pathological examination revealed mild hyperkeratosis of the epidermis, nodular inflammatory lesions in the dermis, epithelioid granuloma, and inflammatory cell infiltration with lymphocytes and histiocytes around skin appendages and blood vessels. Alcian blue staining, acid fast staining, silver staining and periodic acid-Schiff staining were negative. The patient was diagnosed with Morbihan disease. She was treated with prednisone acetate and tripterygium wilfordii polyglycoside tablets for 4 mo, and the edema was slightly reduced, but transaminase levels were significantly increased. Compound glycyrrhizin capsules were administered for liver protection for 1 mo; however, facial edema did not significantly improve and transaminase levels continued to increase. Total glucosides of paeony capsules were then administered for 4 mo, and transaminase level returned to normal and the patient's facial edema disappeared completely.
Total glucosides of paeony has a remarkable effect in Morbihan disease, without adverse reactions.
莫尔比昂病是一种罕见的皮肤疾病，其特征为面部上三分之二出现非凹陷性水肿和红斑。在严重病例中，眼眶和面部轮廓改变可能影响视野，且该病尚无标准治疗指南。据报道，现有治疗方法存在用药周期长、停药后易复发以及多种不良反应等问题。
一名55岁中国女性因面部上三分之二出现非凹陷性水肿和红斑5个月就诊于我院。体格检查显示面部上部有明显水肿和红斑。边界不清，病变质地硬且非凹陷性，触诊时有明显浸润。病理检查显示表皮轻度角化过度，真皮有结节性炎性病变、上皮样肉芽肿，皮肤附属器和血管周围有淋巴细胞和组织细胞浸润。阿尔辛蓝染色、抗酸染色、银染色和过碘酸希夫染色均为阴性。该患者被诊断为莫尔比昂病。她接受醋酸泼尼松和雷公藤多苷片治疗4个月，水肿稍有减轻，但转氨酶水平显著升高。给予复方甘草酸苷胶囊保肝治疗1个月；然而，面部水肿未明显改善，转氨酶水平持续升高。随后给予白芍总苷胶囊治疗4个月，转氨酶水平恢复正常，患者面部水肿完全消失。
白芍总苷治疗莫尔比昂病疗效显著，且无不良反应。
