Morbihan病

报告1例Morbihan病。患者男,51岁。面部反复水肿性红斑4年余,加重4个月。皮肤科检查:额部(眉间)、上眼睑、鼻部、颧部可见水肿性红斑,其上散在分布约粟米大红色丘疹;胸部及背部可见片状境界不清的红斑。皮损组织病理检查:表皮萎缩,基底细胞空泡变性;真皮浅层可见扩张的管腔,胶原间明显水肿,血管周围可见淋巴组织细胞浸润。诊断:Morbihan病。予口服羟氯喹及异维A酸治疗,未见明显疗效。

Morbihan disease misdiagnosed as senile blepharoptosis and successfully treated with short-term minocycline and ketotifen: A case report

BACKGROUND Morbihan disease is a rare skin condition with diagnostic and therapeutic challenges.Facial nonpitting erythematous edema is usually considered to be a characteristic manifestation and diagnostic clue for the Morbihan disease.Treatment of Morbihan disease remains a dilemma due to its long course,poor response,and high recurrence rate.CASE SUMMARY We report the case of a 69-year-old man with Morbihan disease.The patient presented ptosis as the first and main symptom.There was no obvious edema or other skin lesions.The patient was misdiagnosed with senile blepharoptosis based on eyelid performance and no treatment was administered to him.After continuous progressive asthenia of eye-opening and ptosis for more than one year,a skin biopsy was recommended.Histopathological analysis showed edema in the dermis,lymphatic hyperplasia and dilatation,and perivascular lymphocytic infiltration.An obvious increase in toluidine blue-stained mast cells was observed.The patient was finally diagnosed with Morbihan disease.Minocycline and ketotifen were prescribed based on the increase of mast cells in skin tissue slices.The patient experienced rapid relief seven days later and complete remission after 40 d from the commencement of the treatment.CONCLUSION Ptosis without obvious swelling could be the only or main clinical manifestation of Morbihan disease in rare conditions.An increase of mast cells was an important therapeutic clue to the rapid and remarkable efficiency of the combination therapy of minocycline and antihistamine.

Morbihan病一例

患者,男,76岁。面部红斑、肿胀1年。皮肤科查体:中上面部对称性、弥漫性分布的红斑及非凹陷性水肿,皮损边界不清。皮损组织病理学示表皮大致正常,真皮浅层血管周围淋巴细胞为主的炎性细胞浸润。结合临床特点及组织病理学及特殊染色表现诊断为Morbihan病,目前长期随访中。

Successful treatment of Morbihan disease with total glucosides of paeony:A case report

BACKGROUND 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.CASE SUMMARY 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.CONCLUSION Total glucosides of paeony has a remarkable effect in Morbihan disease,without adverse reactions.

Morbihan病国内首报

报告国内首例Morbihan病。患者男,40岁。面部红斑及肿胀3年。皮肤科检查:上面部弥漫性淡红斑和以眼眶为中心的非凹陷性水肿,皮损边界不清。皮损组织病理检查:表皮大致正常,真皮浅层毛细血管扩张,血管和附属器周围组织细胞和以淋巴细胞为主的炎性细胞浸润,可见少量中性粒细胞。阿辛蓝染色阴性。诊断:Morbihan病。

Progressive disfiguring facial masses with pupillary axis obstruction from Morbihan syndrome:A case report

BACKGROUND Morbihan syndrome is a rare illness,which presents with recurrent pitting edema on the upper two thirds of the face causing facial contour deformities,and Morbihan syndrome obstructing the pupillary axis with ptosis was seldomly reported.CASE SUMMARY A 59-year-old woman presented with a 15-year history of facial swelling that progressively invaded bilaterally from the inner canthus and eyelids outwards.Imaging examination indicated that the bilateral periorbital and the left temporal soft tissues had swelling without cranium invasion.Histopathological analysis showed a large amount of lymphocyte infiltration,and immunohistochemistry showed positive expression of CD68 in clear-cut granulomas and D2-40 in the lymphatic endothelium.Finally,the clinical diagnosis of Morbihan syndrome was confirmed.CONCLUSION Morbihan syndrome is an infrequent and refractory disease,which is characteristic with recurrent woody facial edema on the upper two thirds of the face.Solid facial edema is persistent and non-pitting,causing facial contour deformities and even vision field impairment.The diagnosis of Morbihan syndrome depends on clinical features,imaging information,and pathology.Blepharoplasty is optional to improve the visual field for these patients with severe pupillary axis obstruction.

Morbihan病的研究进展

Morbihan病是一种罕见的以慢性面部持续性水肿为主要特征的皮肤疾病,其缺乏特异性的实验室诊断指标及组织病理学的改变,且发病病因尚不完全明确,至今尚无统一的治疗方法,未来仍需进一步研究。该文就Morbihan病的研究进展作一综述。

Morbihan综合征1例

患者男,20岁,面部红斑、丘疹5年,肿胀4余年。皮肤科情况:全脸非凹陷性水肿,触之有浸润感;面部散在红斑、丘疹。皮损组织病理示:表皮基底层灶性空泡变性,真皮全层小血管周围较多淋巴细胞浸润,真皮胶原间疑似黏液沉积。阿辛蓝染色可见真皮胶原间弥漫性黏液沉积。弹力纤维染色示:真皮弹力纤维数量减少,弹力纤维碎片化。诊断:Morbihan综合征。

Heavy Metals Concentration in Sediments of South Brittany Waters, France: An Ecological Risk Assessment Approach

Distribution patterns of selected heavy metals content in sediments from the Bay of Quiberon and Gulf of Morbihan were studied to understand the current heavy metals contamination due to urbanization and mariculture activities in the coastal area. Therefore, a survey was conducted and 196 sediments collected were characterized for heavy metals content using Inductively Coupled Plasma-Mass Spectrometry (ICP-MS) after mix acid digestion process. The distribution maps of the concentrations of the heavy metals studied were produced as an isopleth map based on data interpolation by the ArcGIS software application. The association with the adverse effects on aquatic organisms was determined by the classification of the sediment according to the sediment quality guidelines. Therefore, two approaches were employed namely: direct comparison with Sediment Quality Guidelines (SQGs) by USEPA (United States Environmental Protection Agency) and comparison with other numerical SQGs, threshold effect level/probable effect level, and effect range low/effect range medium. In order to estimate the effect of multiple contaminations of heavy metals, the mean-ERM-quotient was calculated at each sampling point.