PDE-4抑制剂治疗难治性Morbihan病1例

Intractable Morbihan Disease Successfully Treated with PDE-4 Inhibitor:A Case Report

患者男,18岁。面部肿胀逐渐加重不伴痛痒感3年。曾于多家医院反复诊治,先后口服多西环素、异维A酸等药物效果不佳。皮损组织病理示:毛囊上皮细胞轻度水肿,组织略疏松。真皮浅层血管、毛囊周围少许淋巴细胞浸润,阿新兰染色:真皮内胶原间少许黏蛋白沉积。免疫组织化学染色:真皮浅中层D2-40染色可见明显淋巴管扩张,CD31血管内皮阳性表达。结合临床诊断为Morbihan病,给予PDE-4抑制剂阿普米司特口服,治疗1周开始起效,2周后肿胀明显缓解,1个月后红斑、丘疹明显消退。

The 18-year-old male patient presented with facial swelling that had been worsening over the past three years.The swelling was not accompanied by any pain or itching.The patient had previously visited other hospitals,where he was treated with antibiotics,isotretinoin and other drugs but had not seen any significant improvement.Histopathology showed that the hair follicle epithelial cells were slightly edematous,and there was some lymphocyte infiltration around superficial dermal blood vessels and hair follicles.Ashin blue staining showed a small amount of mucin deposited between collagen in the dermis.Immunohistochemical staining of superficial dermis with D2-40 antibody showed obvious lymphatic dilatation,and CD31-positive stain in vascular endothelium.Based on these finding and the patient′s symptoms,the clinical diagnosis was Morbihan syndrome.After taking PDE-4 inhibitor Apremilast,the patient experienced relief after a week,significant reduction in swelling after two weeks,and decrease in erythema and papules after one month.