Subepidermal Autoimmune Bullous Disease Associated With Monoclonal Gammopathy of Unknown Significance and Unexpected Positive Direct Nikolsky Phenomenon:A Case Report  

作　　者：Gamze Tas-Aygar Müzeyyen Gonül Aysun Gokce Münire Cagin 

机构地区：[1]Dermatology Clinic,University of Health Sciences,Diskapi Yildirim Beyazit Training and Research Hospital,Ankara 06110,Turkey [2]Pathology Clinic,University of Health Sciences,Diskapi Yildirim Beyazit Training and Research Hospital,Ankara 06110,Turkey

出　　处：《International Journal of Dermatology and Venereology》2021年第1期45-47,共3页国际皮肤性病学杂志（英文）

摘　　要：Introduction:Numerous dermatoses associated with monoclonal gammopathy have been reported in the literature.Subepidermal autoimmune bullous diseases(SABD)are one of them which were not common.Case presentation:A 68-year-old male patient was admitted to our clinic with erosions on the oral mucosa,tense blisters,erosions,and ulcers on the trunk and extremities.Subepidermal vesicle formation was detected in the skin biopsy.Clinical examination revealed positivity for the Nikolsky phenomenon.The disease was unresponsive to conventional treatments and dysphagia and hoarseness occurred.The patient was screened for malignancy due to his unresponsiveness to the treatments and his severe oral mucosal involvement.Ig-G MGUS was detected in the patient.Discussion:The Nikolsky sign is an indicator of acantholysis and is known as a specific finding for pemphigus.However,when we look at gammopathy-associated autoimmune bullous dermatoses,skin fragility has been reported in cases.However,the meaning of fragility is not explained.The diagnosis of all these patients was Ig-M MGUS.Our patient was presented because of non-IgM MGUS,direct Nikolsky positivity,and severe mucosal involvement.Conclusion:Nikolsky positivity may be a clue for gammopathy-related subepidermal autoimmune bullous diseases.

关 键 词：subepidermal autoimmun bullous diseases GAMMOPATHY MGUS Nikolsky phenomenon case report 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]