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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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