Mucous membrane pemphigoid

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.

Management strategy

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.

Interventions for mucous membrane pemphigoid and epidermolysis bullosa aquisitia.

Kirtshig G, Murrell D, Wojnarowska F, Khumalo N. Cochrane Database Syst Rev 2003;CD004056.

The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment and prognostic indicators.

Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, et al. Arch Dermatol 2002; 138: 370–9.

Specific investigations

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

First-line therapies

Topical corticosteroids	C
Dapsone	C
Anti-inflammatory antibiotics	D
Systemic corticosteroids	C
Cyclophosphamide	C

Mild disease

The management of oral mucous membrane pemphigoid with dapsone and topical corticosteroid.

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.

Treatment of severe erosive gingival lesions by topical application of clobetasol propionate in custom trays.

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.

Treatment-resistant or severe disease

Dapsone efficacy and adverse events in the management of mucous membrane pemphigoid.

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.

The management of oral mucous membrane pemphigoid with dapsone and topical corticosteroid.

Arash A, Shirin L. J Oral Pathol Med 2008; 37: 341–4.

All 15 patients had a significant response to topical corticosteroids and dapsone 25–100 mg daily. Ten had complete resolution.

Dapsone therapy of cicatricial pemphigoid.

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.

Antiepiligrin cicatricial pemphigoid of the larynx successfully treated with a combination of tetracycline and niacinamide.

Sakamoto K, Mori K, Hashimoto T, Yancey KB, Nakashima T. Arch Otolaryngol Head Neck Surg 2002; 128: 1420–3.

Successful therapy with tetracycline and nicotinamide in cicatricial pemphigoid.

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).

Systemic minocycline as a therapeutic option in predominantly oral mucous membrane pemphigoid: a cautionary report.

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.

Combination therapy with nicotinamide and tetracyclines for cicatricial pemphigoid: further support for its efficacy.

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.

Mucous membrane pemphigoid. Treatment experience at two institutions.

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.

Cicatricial pemphigoid: a re-evaluation of therapy.

Nayar M, Wojnarowska F. J Dermatol Treat 1993; 4: 89–93.

Five of 15 patients with generalized MMP benefited from prednisolone 40–60 mg/day, but it was ineffective in oral disease.

Treatment with systemic corticosteroids seems helpful at doses of prednisolone 0.5–2 mg/kg/day. Most studies used a combination of systemic steroids and a steroid-sparing drug; a small randomized trial supported combination treatment in ocular MMP (see below).

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Tags: Treatment of Skin Disease Comprehensive Therapeutic Strategies

Aug 7, 2016 | Posted by admin in Dermatology | Comments Off

Interventions for mucous membrane pemphigoid and epidermolysis bullosa aquisitia.

The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment and prognostic indicators.

The management of oral mucous membrane pemphigoid with dapsone and topical corticosteroid.

Treatment of severe erosive gingival lesions by topical application of clobetasol propionate in custom trays.

Dapsone efficacy and adverse events in the management of mucous membrane pemphigoid.

The management of oral mucous membrane pemphigoid with dapsone and topical corticosteroid.

Dapsone therapy of cicatricial pemphigoid.

Antiepiligrin cicatricial pemphigoid of the larynx successfully treated with a combination of tetracycline and niacinamide.

Successful therapy with tetracycline and nicotinamide in cicatricial pemphigoid.

Systemic minocycline as a therapeutic option in predominantly oral mucous membrane pemphigoid: a cautionary report.

Combination therapy with nicotinamide and tetracyclines for cicatricial pemphigoid: further support for its efficacy.

Mucous membrane pemphigoid. Treatment experience at two institutions.

Cicatricial pemphigoid: a re-evaluation of therapy.

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Specific investigations

First-line therapies

Mild disease

Treatment-resistant or severe disease

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