Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita

Cyclophosphamide combined with corticosteroids may be effective for mucous membrane pemphigoid in patients with eye involvement. However, mucous membrane pemphigoid with mild to modest inflammatory activity responds to dapsone in most participants and may therefore be best treated with dapsone due to its lower side effect profile compared to cyclophosphamide.

A Cochrane review (abstract , review ) included 2 studies with a total of 64 patients with severe eye involvement.

In the first trial, involving 24 participants, cyclophosphamide 2 mg/kg/day in combination with prednisone starting at 1 mg/kg/day and tapering was superior to prednisone alone (1 mg/kg/day) after 6 months of treatment. All 12 participants responded well to cyclophosphamide plus prednisone versus a good response in only 5 of 12 participants treated with prednisone (RR 2.40, 95% Cl 1.23 to 4.69). In the second trial, involving 40 participants, all 20 participants treated with cyclophosphamide (2 mg/kg/day) responded well after three months of treatment, but only 14 of 20 participants responded to treatment with dapsone (2 mg/kg/day) (RR 1.43, 95% Cl 1.07 to 1.90). All non-responders had severe inflammatory activity.Hair loss and suppression of the red and white blood cells were common adverse events in the cyclophosphamide groups.

No randomised controlled trials of treatments for epidermolysis bullosa acquisita were identified.

Comment: The quality of evidence is downgraded by imprecise results; few patients and wide confidence intervals.

Ədəbiyyat

1. Kirtschig G, Murrell D, Wojnarowska F et al. Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita. Cochrane Database Syst Rev 2003;(1):CD004056. . [Assessed as up-to-date: 23 JAN 2006]