한국인 선IgA물집피부병의 임상연구

온혜랑 ( Hye Rang On ),김수찬 ( Soo Chan Kim ) 대한피부과학회 2015 大韓皮膚科學會誌 Vol.53 No.7

Background: Linear IgA bullous dermatosis (LABD) is an autoimmune, chronic bullous disease characterized by sub-epithelial bullae with linear IgA deposits along the basement membrane. LABD primarily affects young children and adults. There has been no study on LABD in Korea to date. Objective: The purpose of this study was to evaluate the clinical features, laboratory examinations, treatments, and outcomes of Korean LABD patients. Patient characteristics including age at disease onset, gender, medical associations, medications, immunofluorescence findings, disease duration, treatment, and outcome were analyzed. Methods: A retrospective analysis was conducted on 16 LABD patients diagnosed at Gangnam Severance Hospital between 1999 and 2014. Results: A total of 16 LABD patients were included in the study, 5 children and 11 adults. The mean ages at disease onset in children and adults were 3.2 and 41 years, respectively. Eighty percent of children with LABD showed complete remission. In adults, partial remission was achieved in 36.4%, and complete remission in 54.5% of patients. Two patients were diagnosed with drug-induced LABD, and 2 with ulcerative colitis-associated LABD. Conclusion: Our report differed from previous reports in that all 5 children with LABD were male, and the incidence in adults was higher in females than males. Most patients responded well to dapsone and oral prednisolone. Since LABD is rare and can be misdiagnosed as impetigo or bullous pemphigoid, diagnosis by immunofluorescence microscopy is necessary for proper treatment to attain disease remission. (Korean J Dermatol 2015;53(7):515∼522)

Vancomycin-induced Linear IgA Bullous Dermatosis: A Case Report and Review of the Literature

Min Ju Kang,Hyung Ok Kim,Young Min Park 대한피부과학회 2008 Annals of Dermatology Vol.20 No.2

Linear IgA Bullous Dermatosis

김홍직(Hong Jig Kim),김영근(Young Keun Kim),표형찬(Hyung Chan Pyo),이창우(Chang Woo Lee) 대한피부과학회 1987 대한피부과학회지 Vol.25 No.4

A case of linear IgA bullous dermatosis reported. A 32-year-old male with pruritic, papulovesicular eruptions on the whole body of 2 months' duration was examined. He had no history of diarrhea or fou]smellirig stool even though his diet cnntained gluten. A skin biopsy revealed subepidennal vesicles and papillary microabscesses infiltrated by many neutrophils and some eosinophils. Direct irnmunofluorescent examination of the perilesional skin showed a linear deposition of IgA along the basement mernbrane zone. The lesions responded rapidly to oral dapsone and topical steroid therapy but recurred whenever the oral mcdication was discontinued.

Successful treatment of linear IgA dermatosis with rituximab therapy

( Seung-ju Lee ),( Jong Hoon Kim ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.2

Background: Rituximab, a monoclonal antibody against CD20, is widely used in autoimmune blistering diseases. However, the therapeutic value of rituximab in linear IgA dermatosis (LAD) is uncertain because there are only a few case reports on successful use of rituximab in recalcitrant LAD. Objectives: To evaluate the efficacy of rituximab for the treatment of recalcitrant LAD. Methods: Three patients were diagnosed as LAD based on the clinical, histological, and immunofluorescence studies. All patients had been refractory to high-dose systemic corticosteroid (16~24 mg/day of methylprednisolone) with other conventional agents over the past 2 months. Patient #1 and #2 were treated with two 1000 mg infusions of rituximab separated by 2 weeks, and patient #3 only received 1st infusion due to pneumonia during the rituximab treatment. Results: Follow-up periods were 6 months in patient #1, 4 months in patient #2, and 11 months in patient #3. Remission with minimal therapy (8mg/day of methylprednisolone) was achieved 2 months after rituximab therapy in patient #1 and #2, and 5 months after rituximab therapy in patient #3. Blister formation did not appear during 6-month and 3-month follow-up period in patient #1 and #2, respectively. Patient #3 experienced relapse 10 months after rituximab therapy. Conclusion: Rituximab should be considered as a feasible treatment in severe cases of LAD refractory to conventional treatments.

유아에서 발생한 선상 IgA 수포성 피부병

박수홍 ( Soo Hong Park ),정문철 ( Moon Cheol Jeong ),김계정 ( Kea Jeung Kim ),강형재 ( Hyung Jai Kang ) 대한피부과학회 1998 대한피부과학회지 Vol.36 No.2

Linear IgA bullous dermatoses(LABD) occurring in childhood is considered to be a childhood counterpart of LABD,rather than chronic bullous dermatosis of childhood. This disease characteristically involves the perioral area,lower trunk,pelvic region and the lower extremities. Immunopathologically,it is characterized by the linear deposits of IgA at the dermoepidermal junction of the perilesional skin. A 20-month-old female infant presented with multiple pruritic tense bullae on the face, lower trunk, both extremities and pelvic region of 2 weeks duration. She had no oral lesions or any specific signs or symptoms referable to the disease of the gastrointestinal tract. A skin biopsy revealed subepidermal blisters infiltrated with many neutrophils and a few eosinophils. Direct immunofluorescence demonstrated linear deposition of IgA at the dermoepidermal junction. An indirect immunofluorescence study using NaC1-split skin as the substrate revealed fluorescence for IgA in a linear pattern on the roof of the split at a titer of 1:40. The clinical diagnosis of LABD of childhood was confirmed and we started with a combined remedy of dapsone and corticosteroids; 10 weeks later almost all of the lesions had disappeared. (Korean J Dermatol 1998;36(2): 304-307)

Captopril에 의해 유발된 것으로 생각되는 선상 IgA 수포성 피부병

권연숙 ( Yeon Sook Kwon ),오상호 ( Sang Ho Oh ),정진영 ( Jin Young Jung ),류동진 ( Dong Jin Ryu ),이주희 ( Ju Hee Lee ) 대한피부과학회 2007 대한피부과학회지 Vol.45 No.11

Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepidermal bullous disease characterized by a linear pattern of IgA deposition along the basement membrane zone. The etiology of LABD is unknown but a minority of cases are drug-induced. Although vancomycin is the agent most commonly implicated in the pathogenesis of drug-induced LABD, association with other agents including phenytoin, piroxicam, captopril, diclofenac sodium and cyclosporine have also been reported. We describe a case of linear IgA bullous dermatosis which probably developed after captopril treatment in a 51-year-old male patient with infective endocarditis. (Korean J Dermatol 2007;45(11):1202∼1205)

Piperacillin-Tazobactam-Induced Linear IgA Bullous Dermatosis Supported by a T-Cell Activation Assay

( Yi-hsin Ho ),( Yun-wen Chiu ),( Han-nan Liu ) 대한피부과학회 2018 Annals of Dermatology Vol.30 No.5

Linear immunoglobulin (Ig) A bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disease characterized by linear IgA deposits at the basement membrane zone visualized with direct immunofluorescence (DIF). Most cases of LABD are idiopathic, but some are drug-induced with vancomycin being the most common causative agent. We herein report a patient presenting with blisters and erosive lesions, primarily in the intertriginous and flexor areas, consistent with a diagnosis of piperacillin-tazobactam-induced LABD based on the patient’s clinical course and histopathology, DIF, and in vitro T-cell activation assay (TAA) findings. Only one case of piperacillin-tazobactam-induced LABD has been previously reported. In addition to its rarity, our case was also unique in that the skin lesions occurred in the intertriginous and flexor areas, uncommon locations for typical adult patients with LABD, and TAA strongly suggested an association with the causative drug. (Ann Dermatol 30(5) 588∼591, 2018)

Linear IgA bullous dermatosis associated with ulcerative colitis

( Tae Hyung Kim ),( Soo Chan Kim ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Linear IgA bullous dermatosis (LABD) is an autoimmune bullous disease characterized by subepidermal bullae, with continuous linear IgA deposit along the basement membrane zone. A correlation of LABD with inflammatory bowel disease (IBD), particularly ulcerative colitis (UC) is well documented. Association between the two diseases may be related to the exposure of the immune system to autologous antigens in the bowel, which are then recognized in the skin. A 45-year-old woman presented with a 2-week history of erythematous vesicles and patches with erosions on the trunk. She has suffered from ulcerative colitis since 5 years ago. Histology revealed subepidermal blister with neutrophils and eosionphils infiltration. Direct immunofluorescence depicted linear deposition of IgA along the basement membrane zone. Indirect immunofluorescence showed circulating IgA anti-basement membrane zone antibodies binding to the epidermal side of salt-split skin. The skin lesions have been well controlled with methylprednisolone and dapsone therapy.

A case of linear IgA bullous dermatosis induced by vancomycin

( Ye-jin Lee ),( Hyo-eun Kwon ),( Jong-kil Seo ),( In-hye Kang Min ),( Kyung Shin ),( Ki-heon Jeong ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepidermal blistering disorder, which is usually idiopathic but can be rarely induced by medications or infectious causes. Vancomycin considered as a prevalent choice for the treatment of serious infections is known as the most well known cause of drug-induced linear IgA bullous dermtosis. Early diagnosis of vancomycin induced LABD is the key to appropriate management. A 67-year-old male was consulted to our department because of sudden onset of asymptomatic blisters on the whole body. These lesions first appeared on the right arm 2 days ago. He underwent spine surgery and treated with intravenous vancomycin IV because of infection. Skin biopsy from the arm demonstrated subepidermal blister and infiltration of neutrophils. Direct immunofluorescence of the specimen taken from perilesional normal skin showed linear deposits of IgA along the basement membrane zone. Skin lesions improved after discontinuation of vancomycin. We herein report a rare case of vancomycin-induced LABD.

약제에 의한 선상 IgA 수포성 피부병 1 례

함상희,하석준,박영민,조상현,김진우,조백기 ( Sang Hee Ham,Seog Jun Ha,Young Min Park,Sang Hyun Cho,Jin Wou Kim,Baik Kee Cho ) 대한천식알레르기학회 1998 천식 및 알레르기 Vol.18 No.3

Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease characterized on direct immunofluorescence of perilesional skin by the linear deposition of IgA at the basement membrane zone. The pathogenesis of LABD is not known, but rar ely some cases of LABD have been reported in association with drug exposure. We report a case of drug-induced LABD showing linear deposits of IgA and IgG at the basement membrane zone on direct immunofluorescence of perilesional skin as well as typical clinical and histologic characteristics, although the causative agent could not be specified.