Published online Jul 6, 2022. doi: 10.12998/wjcc.v10.i19.6688
Peer-review started: January 13, 2022
First decision: April 8, 2022
Revised: April 15, 2022
Accepted: May 17, 2022
Article in press: May 17, 2022
Published online: July 6, 2022
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.
Core Tip: Morbihan disease is a rare skin disease characterized by non-pitting edema and erythema of the upper two-thirds of the face. The orbital and facial contour changes can affect the field of vision and cause disfiguring damage if not treated in time. The specific pathogenesis is unclear, and there is no standard treatment guideline for this disease. Thus, it is necessary to identify a safe and effective treatment. We treated a case of Morbihan disease with total glucosides of paeony and achieved very good results with no adverse reactions. The treatment of Morbihan disease with total glucosides of paeony has not previously been reported.