Understanding Mucous Membrane Pemphigoid

Mucous membrane pemphigoid is a rare chronic autoimmune disorder that affects the mucous membranes of the body, including the vagina, with blisters.¹ The eyes and mouth are the most often affected, but the nose, throat, vulva and anus may be affected.

Symptoms of mucous membrane pemphigoid

Symptoms vary between people
Blistering lesions that eventually heal, sometimes scarring
Often appearing on head and neck
Recurrent blistering
Red and blistered mouth, gums, palate
Tender gums, may bleed easily
May affect oesophagus and larynx
Difficulty swallowing
Conjunctivitis, one eye at a time
Skin may be involved with blisters or plaques that bleed or itch
Hyperpigmentation
Alopecia
Frequent remit-relapse episodes
Can affect the vulva

Causes of mucous membrane pemphigoid

The cause is unknown, but it is an autoimmune disorder. Antibodies attack the basement membrane zone of the epithelium, allowing the skin to lift off and create the blisters.² Genetics may be involved. Prescription drugs in some cases are responsible. Damage to skin may trigger the condition.³

Women are affected twice as often as men, with the average age of onset after 60, but it can appear at any age. It may be underdiagnosed.

What else could it be?

Bullous pemphigoid
Epidermolysis bullosa acquista
Pemphigus vulgaris
Lichen planus
Lichen sclerosus
Brunsting-Perry syndrome
Stevens-Johnson syndrome
Erythema multiforme minor
Paraneoplastic pemphigus
Linear IgA bullous dermatosis

Treatment for mucous membrane pemphigoid

Individualised treatment is required, depending on the area affected and how severe the condition is. It is likely to involve a team of specialists to deal with each area affected, for example, a dentist, dermatologist, ophthalmologist, otolaryngologists and a gynaecologist.

Treatment may include antibiotics, steroids or immunosuppressive drugs.⁴ Surgery may be involved in some cases.

References

1.
Du G, Patzelt S, van Beek N, Schmidt E. Mucous membrane pemphigoid. Autoimmunity Reviews. Published online April 2022:103036. doi:10.1016/j.autrev.2022.103036
2.
Murrell DF, Marinovic B, Caux F, et al. Definitions and outcome measures for mucous membrane pemphigoid: Recommendations of an international panel of experts. Journal of the American Academy of Dermatology. Published online January 2015:168-174. doi:10.1016/j.jaad.2014.08.024
3.
Deotto M, Spiller A, Sernicola A, Alaibac M. Bullous pemphigoid: An immune disorder related to aging (Review). Exp Ther Med. Published online November 15, 2021. doi:10.3892/etm.2021.10972
4.
Buonavoglia A, Leone P, Dammacco R, et al. Pemphigus and mucous membrane pemphigoid: An update from diagnosis to therapy. Autoimmunity Reviews. Published online April 2019:349-358. doi:10.1016/j.autrev.2019.02.005