Epidermolysis bullosa acquisita: A retrospective clinical analysis of 30 cases

Jong Hoon Kim, Yeon Hee Kim, Soo Chan Kim

Research output: Contribution to journalArticle

55 Citations (Scopus)

Abstract

Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune blistering disorder caused by autoanti-body 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-li-ke (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 met-hylprednisolone, dapsone and colchicine. No signifcant differences in time to remission were identifed 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).

Original languageEnglish
Pages (from-to)307-312
Number of pages6
JournalActa dermato-venereologica
Volume91
Issue number3
DOIs
Publication statusPublished - 2011 May 1

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Epidermolysis Bullosa Acquisita
Bullous Pemphigoid
Methylprednisolone
Linear IgA Bullous Dermatosis
Collagen Type VII
Dapsone
Colchicine
Age of Onset
Mucous Membrane
Incidence

All Science Journal Classification (ASJC) codes

  • Dermatology

Cite this

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abstract = "Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune blistering disorder caused by autoanti-body 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-li-ke (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 met-hylprednisolone, dapsone and colchicine. No signifcant differences in time to remission were identifed 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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Epidermolysis bullosa acquisita : A retrospective clinical analysis of 30 cases. / Kim, Jong Hoon; Kim, Yeon Hee; Kim, Soo Chan.

In: Acta dermato-venereologica, Vol. 91, No. 3, 01.05.2011, p. 307-312.

Research output: Contribution to journalArticle

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