Mucous membrane pemphigoid
Last Updated: 2023-09-28
Author(s): Navarini A.A.
Known as mucosamembranous pemphigoid (MMP) or scarring pemphigoid. Other names include: Cicatricial pemphigoid, Benign mucosal pemphigoid, Ocular pemphigus, Dermatitis pemphigoides mucocutanea chronica, Ocular mucous membrane pemphigoid, Ocular mucosal pemphgoid, Ocular MMP, Dermatite bulleuse mucosynéchiante, Pemphigus conjunctivae, Pemphigus oculaire, Pemphigus ocular, Scarring pemphigoid, Scarring mucous membrane pemphigoid, Benign mucous membrane pemphigus, Syndromes muco-oculo-épithélial, Scarring pemphigoid and Desquamative gingivitis.
First described by Lortat-Jakob in 1958, but already mentioned by Wichmann in 1793.
MMP is characterised as a rare autoimmune disease that leads to blistering and scarring of mucous membranes such as the mouth, eyes, throat, oesophagus and reproductive organs.
Bullous pemphigoid is a very closely related disease. An interesting entity that may be in between is Brunsting Perry's scarring pemphigoid, which involves the scalp.
Based on immunohistology, four types can be distinguished:
- Anti-epileprin Cicatricial Pemphigoid.
- Ocular cicatricial pemphigoid characterised by autoantibodies against β4-integrin and α6β4-integrin.
- Cases with autoantibodies against BP 180, showing both skin and mucosal lesions.
- Anti-laminin 332 mucosal pemphigoid occurring in 20-30% of patients, with about 35% associated with cancer.
Although MMP is rare and has no ethnic tendency, the incidence in Western Europe is 0.08-0.2 per 100,000 persons annually. It manifests predominantly in seniors, especially women aged 60-80 years. Earlier onset in childhood is uncommon.
Distribution of affected areas:
- Mouth: 85%
- Eyes: 64%
- Throat: 19%
- Pelvic organs: 17%
- Nose: 15%
- Larynx: 8%
- Anal region: 4%
- Oesophagus: 4%
Most often, the disease first manifests itself as therapy-resistant, erosive inflammation in the mouth. Here, 100% of cases are affected with recurrent, painful ulcers in the oral mucosa. In addition, 80% of patients experience recurrent conjunctivitis. The dangerous thing about this disease is the slow destruction, which can lead to adhesions on the eye, symblepharon and so on. Less frequently, the nasal mucous membranes are also involved, in about 40 %. The genital mucous membranes can also be involved.
A regular mistake is the interpretation of the disease by the dermatologist as too harmless, and thus the delay of aggressive immunosuppression.
Circulating antibodies against the basement membrane are not the rule. However, IgA autoantibodies against the desmosomal protein BP 180 (BP230) and a specific 168 kD antigen of the buccal mucosa are not uncommon. In patients with only eye involvement, antibodies against a 45 kDa antigen can be detected.
A striking feature is the formation of subepidermal blisters surrounded by an inflammatory reaction involving mainly neutrophils and eosinophilic granulocytes.
Treatment is challenging because of the ongoing course of the disease. Immunosuppressants and high-dose steroids, such as azathioprine, methotrexate or mycophenolate mofetil, are often used.
Serious consequences include blindness due to scarring of the conjunctiva and tightness in the larynx, which leads to breathing difficulties.
For localised scarring pemphigoid, the use of glucocorticoids is the first choice. In a mild course of the disease, local applications such as eye ointments containing dexamethasone or hydrocortisone are sufficient. Alternating corticosteroids with corticosteroid-free ophthalmic preparations or vasoconstrictive ophthalmic medications, as well as performing ocular irrigation, may also be beneficial. If the conjunctiva is more severely affected, ciclosporin A eye drops are advisable. If there is evidence of bacterial over-infection, antibiotic eye drops should be considered
For oral discomfort, especially painful mucosal defects, local glucocorticoid applications in the form of adhesive oral gels or solutions are useful. If the treatment does not work, adhesive pastes with ciclosporin A or tacrolimus ointments can be useful. Furthermore, the use of gentle mucosal agents or astringent preparations is recommended.
Scarring mucous membrane pemphigoid is often a therapeutic challenge and often requires intensive and long-term treatment with high doses of immunosuppressants. Especially when the eye is involved, a combined treatment of glucocorticoids and azathioprine is suggested. The starting doses for prednisone and azathioprine are 2.0 mg/kg body weight each. There are also alternatives such as cyclophosphamide, ciclosporin A and mycophenolate mofetil. Dapsone is rarely considered due to unconvincing results. Clinical trials with etanercept have been noted. There are also reports of successful use of rituximab infusions. In case of non-response to therapy, a combination treatment of dapsone, glucocorticoids and further immunosuppressants could be helpful.
In addition to medication, surgical procedures may be necessary, especially in the eye area. These include separating adhesions between the bulbar and tarsal conjunctiva or removing scars on the eyelid. There are various surgical options, but they are not comprehensively presented here.
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