Published on 19/03/2015 by admin
Filed under Dermatology
Last modified 19/03/2015
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Caroline P. Allen and Vanessa Venning
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Mucous membrane pemphigoid (MMP) formerly known as cicatricial pemphigoid is a heterogeneous group of chronic acquired autoimmune sub-epidermal blistering diseases. It is characterized by blistering and erosions of one or more mucous membranes (eyes, oral mucosa, oesophagus, genitals) and to a lesser extent the skin. This may lead to permanent scarring of the affected area, particularly the conjunctiva.
MMP is a chronic disease and does not generally remit spontaneously; disease activity will fluctuate without treatment, and treatments are disease modifying rather than curative.
Optimal treatment for MMP is unclear as there is little evidence in the form of randomized control trials; international consensus guides treatment strategy, based on the site, severity, extent and rate of progression of disease. Patient co-morbidities must also be considered and a multidisciplinary approach is vital.
Kirtshig G, Murrell D, Wojnarowska F, Khumalo N. Cochrane Database Syst Rev 2003;CD004056.
Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, et al. Arch Dermatol 2002; 138: 370–9.
For diagnosis
Direct immunofluorescence
Indirect immunofluorescence on salt split skin
For treatment
Complete blood count, liver function tests, renal function
Glucose-6-phosphate dehydrogenase activity
Thiopurine methyltransferase
Arash A, Shirin L. J Oral Pathol Med 2008; 37: 341–4.
Five patients with mild MMP had a good response to triamcinolone in orabase.
Gonzales-Moles MA, Ruiz Avila I, Rodriguez-Archilla A, Morales-Garcia P, Mesa-Aguado F, Bascones-Martinez A, et al. Oral Surg Oral Med Oral Pathol Oral Radiol Endodont 2003; 95: 688–92.
Improvement in all 22 patients with oral MMP treated with topical clobetasol propionate with nystatin applied via a gingival tray.
Hegarty AM, Ormond M, Sweeney M, Hodgson T. Eur J Dermatol 2010; 20: 223–4.
Eight out of 20 patients improved with dapsone. One failed to respond and 11 developed side effects necessitating cessation.
All 15 patients had a significant response to topical corticosteroids and dapsone 25–100 mg daily. Ten had complete resolution.
Rogers RS III, Mehregan DA. Semin Dermatol 1988; 7: 201–5.
Seventy-seven patients with oral, ocular or generalized MMP benefited from either 150 mg dapsone per day or 1500–3000 mg sulfapyridine per day for at least 12 weeks.
Treatment with dapsone 50–200 mg/day or sulfapyridine is effective in some patients.
Sakamoto K, Mori K, Hashimoto T, Yancey KB, Nakashima T. Arch Otolaryngol Head Neck Surg 2002; 128: 1420–3.
Kreyden OP, Borradori L, Trüeb RM, Burg G, Nestle FO. Hautarzt 2001; 52: 247–50.
Single case reports of effective treatment with tetracycline and nicotinamide (500–3000 mg/day).
Carrozzo M, Arduino P, Bertolusso G, Cozzani E, Parodi A. Int J Oral Maxillofac Surg 2009; 38: 1071–6.
Seven of nine patients with oral disease responded to minocycline 200 mg/day; however, side effects limited treatment in five patients.
Reiche L, Wojnarowska F, Mallon E. Clin Exp Dermatol 1998; 23: 254–7.
Minocycline 50–100 mg/day combined with 2.5–3 g nicotinamide per day was beneficial in five of eight patients.
Anti-inflammatory antibiotics can be of benefit. Side effects may limit treatment. Nicotinamide may confer additional protection.
Lamey PJ, Rees TD, Binnie WH, Rankin KV. Oral Surg Oral Med Oral Pathol 1992; 74: 50–3.
Fourteen patients with oral MMP were treated with a combination of systemic and topical steroids: eight became asymptomatic, five improved, and one was unchanged.
Nayar M, Wojnarowska F. J Dermatol Treat 1993; 4: 89–93.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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Topical corticosteroids
Dapsone
Anti-inflammatory antibiotics
Systemic corticosteroids
Cyclophosphamide
