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Epidermolysis bullosa acquisita: a retrospective clinical analysis of 30 cases

 Jong Hoon Kim  ;  Yeon Hee Kim  ;  Soo-Chan Kim 
 Acta Dermato-Venereologica, Vol.91(3) : 307-312, 2011 
Journal Title
 Acta Dermato-Venereologica 
Issue Date
Adult ; Aged ; Colchicine/administration & dosage ; Dapsone/administration & dosage ; Drug Therapy, Combination ; Epidermolysis Bullosa Acquisita*/classification ; Epidermolysis Bullosa Acquisita*/diagnosis ; Epidermolysis Bullosa Acquisita*/drug therapy ; Female ; Humans ; Immunosuppressive Agents/administration & dosage ; Kaplan-Meier Estimate ; Male ; Methylprednisolone/administration & dosage ; Middle Aged ; Remission Induction ; Republic of Korea ; Retrospective Studies ; Time Factors ; Treatment Outcome ; Young Adult
Clinical study remission ; epidermolysis bullosa acquisita ; retrospective study
Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune blistering disorder caused by autoantibody production against type VII collagen. The aim of this study was to examine the clinical types, treatments, and outcomes of 30 patients with EBA. In our cohort, the median age of onset was 44.0 years, with a similar incidence for both genders (46.7% male, 53.3% female). The majority of patients had classic type (36.7%) and bullous pemphigoid (BP)-like type (46.7%) EBA. The remaining patients had mucous membrane pemphigoid-like (6.7%), Brunsting-Perry pemphigoid-like (6.7%), and linear IgA bullous dermatosis-like type (3.3%) EBA. All patients were treated initially with a combination of methylprednisolone, dapsone and colchicine. No significant differences in time to remission were identified between patients with classic vs. BP-like EBA. In a second subset analysis of 19 patients, a group treated with high-dose (> 8 mg) methylprednisolone achieved remission earlier (median time to remission: 3 months) than a group treated with low-dose (≤ 8 mg) methylprednisolone (median time to remission: 12 months), irrespective of clinical type (p = 0.003).
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1. College of Medicine (의과대학) > Dept. of Dermatology (피부과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Soo Chan(김수찬) ORCID logo https://orcid.org/0000-0002-2327-4755
Kim, Jong Hoon(김종훈) ORCID logo https://orcid.org/0000-0002-3385-8180
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