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  Vol. 138 No. 3, March 2002 TABLE OF CONTENTS
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  Evidence-Based Dermatology: Original Contribution
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Interventions for Mucous Membrane Pemphigoid/Cicatricial Pemphigoid and Epidermolysis Bullosa Acquisita

A Systematic Literature Review

Gudula Kirtschig, MD; Dédée Murrell, FAAD; Fenella Wojnarowska, DM; Nonhlanhla Khumalo

Arch Dermatol. 2002;138:380-384.

Objective  To identify and critically evaluate evidence from randomized controlled trials for the efficacy of treatments for mucous membrane pemphigoid (MMP)/cicatricial pemphigoid (CP) and epidermolysis bullosa acquisita (EBA).

Search Strategy  Review of MEDLINE from 1966 through March 2000, EMBASE from 1980 through March 2000, and the Cochrane Controlled Trials Register (February 28, 2001) to identify randomized controlled trials for the efficacy of treatments in MMP/CP and EBA.

Selection Criteria  All randomized controlled trials of therapeutic interventions that included patients with MMP/CP or EBA confirmed by immunofluorescence study findings. All age groups were included.

Results  We found 2 small randomized controlled trials of MMP/CP, both conducted in patients with severe eye involvement. We were not able to identify a randomized controlled trial of therapeutic interventions in EBA.

Conclusions  There is evidence from 2 small trials that severe ocular CP responds best to treatment with cyclophosphamide, and mild to moderate disease seems effectively suppressed by treatment with dapsone. No treatment recommendations can be made for EBA because to our knowledge no randomized controlled trials are published. Even though systemic corticosteroids are regarded as the gold standard in the treatment of MMP/CP and EBA, there is poor evidence from the literature that they are the best treatment for these diseases.


From the Department of Dermatology, Oxford Radcliffe Hospital, Oxford, England (Drs Kirtschig and Wojnarowska and Ms Khumalo); the Department of Dermatology, Academisch Ziekenhuis, Vrije Universiteit, Amsterdam, the Netherlands (Dr Kirtschig); the Department of Dermatology, St George Hospital, University of New South Wales, Sydney, Australia (Dr Murrell); and the Department of Dermatology, Observatory, Groote Schuur Hospital, Capetown, South Africa (Ms Khumalo).


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