ConditionSkin, hair & nails
Mucous Membrane Pemphigoid
Mucous membrane pemphigoid (MMP) is a rare, chronic autoimmune disease in which antibodies attack the layer that anchors the surface lining to the tissue beneath, causing blisters and erosions on mucous membranes. The mouth (especially the gums) is affected in most people, and the eyes in many. Unlike most blistering diseases, MMP heals with scarring, which in the eyes can lead to blindness and in the throat to narrowing. Early diagnosis and immune-suppressing treatment prevent much of this damage.
Also called: MMP, cicatricial pemphigoid, ocular cicatricial pemphigoid (eye form), benign mucous membrane pemphigoid
At a glance
- What it is
- An autoimmune blistering disease of mucous membranes that heals with scarring.
- Who gets it
- Usually adults aged 60 to 80; more common in women.
- Most affected sites
- Mouth (about 85%), eyes (about 65%), nose, throat, larynx, esophagus, genitals; skin less often.
- Diagnosis
- Biopsy with direct immunofluorescence showing antibodies along the basement membrane.
- Treatment
- Topical steroids for mild mouth-only disease; dapsone, mycophenolate, rituximab or cyclophosphamide for eye or severe disease.
When to get help
Call 911 or go to the ER
- Noisy breathing, hoarseness with difficulty breathing, or a feeling of throat closing (possible laryngeal involvement). Call 911 or your local emergency number
See a doctor within a day or two
- Red, gritty, painful eyes, or vision getting worse in someone with MMP
- Difficulty swallowing food or liquids, or weight loss
- Painful sores that stop you eating or drinking
Book a routine appointment
- Gum soreness, peeling or bleeding that does not respond to dental care
- Recurrent mouth blisters or sores lasting more than 2 to 3 weeks
- Persistent red, dry or irritated eyes that do not improve with usual treatments
- Nosebleeds and crusting, or genital sores
How mucous membrane pemphigoid works
Mucous membranes line the mouth, eyes, nose, throat and genitals. Their surface cells are anchored to deeper tissue by a structure called the basement membrane zone. In MMP, the immune system produces antibodies (mainly IgG and IgA) against proteins in this zone, including BP180 and laminin 332. The surface separates, forming blisters that quickly break into raw erosions.
Healing triggers scarring (fibrosis), which is what makes MMP dangerous: scarring can close the eyelids onto the eye, narrow the throat or esophagus, and tighten genital tissues.
A subtype with anti-laminin 332 antibodies has an increased association with cancer, so doctors screen for it.
Symptoms by site
| Site | Symptoms |
|---|---|
| Mouth | Painful red, peeling gums (desquamative gingivitis), blisters and erosions on the gums, palate, cheeks; pain with eating and brushing |
| Eyes | Redness, grittiness, burning, tearing, dry eye; later scarring between eyelid and eyeball (symblepharon), inturned lashes, corneal damage, vision loss |
| Nose | Crusting, nosebleeds, blockage |
| Throat and larynx | Sore throat, hoarseness, breathing difficulty |
| Esophagus | Painful or difficult swallowing, strictures |
| Genitals and anus | Painful erosions, scarring, narrowing |
| Skin | Blisters on the scalp, face or upper body in a minority |
Eye disease may begin in one eye and can progress silently, which is why every person with MMP needs regular eye exams even without eye symptoms.
Diagnosis
- Clinical examination of all mucous surfaces, including an eye exam with a slit lamp and often an ENT examination of the nose and larynx.
- Biopsy: a small sample from normal-looking tissue next to a lesion for direct immunofluorescence, which shows a linear band of antibodies at the basement membrane. This is the key test.
- Blood tests: circulating antibodies to BP180, BP230, laminin 332 and others.
- Endoscopy if swallowing is affected.
- Cancer screening for anti-laminin 332 MMP.
Conditions that can look similar include pemphigus vulgaris, oral lichen planus, bullous pemphigoid, and drug reactions.
Treatment
Treatment depends on which sites are involved and how severe the disease is.
Low-risk disease (mouth and skin only)
- potent topical steroid gels or mouth rinses, sometimes in custom dental trays for the gums
- excellent oral hygiene with a soft toothbrush and gentle toothpaste
- dapsone, doxycycline or short courses of oral steroids if needed
High-risk disease (eyes, larynx, esophagus, genitals)
- dapsone for milder eye disease
- mycophenolate mofetil, azathioprine or methotrexate as steroid-sparing medicines
- rituximab and IV immunoglobulin for severe or resistant disease
- cyclophosphamide with steroids for rapidly progressive eye disease
- short-term high-dose oral steroids to gain control
Supportive care
- lubricating eye drops and ointments, treatment of dry eye and eyelash problems
- eye surgery for advanced scarring, ideally only when the disease is inactive
- dilation of esophageal or laryngeal narrowing
- soft, bland diet during flares; avoid crunchy, spicy or acidic foods
- dental care every 3 to 6 months
Complications
- blindness from eye scarring
- airway narrowing
- esophageal stricture and weight loss
- genital scarring and painful intercourse
- side effects of long-term immune suppression, including infections
Outlook
MMP is usually chronic, with periods of remission and relapse. Mouth-only disease generally has a good outlook. Eye and throat disease need close monitoring and stronger treatment. Early diagnosis and adequate immune suppression can stop scarring from progressing, although existing scars do not reverse.
Frequently asked questions
What is mucous membrane pemphigoid?
It is a rare autoimmune disease in which the immune system attacks the tissue that anchors the lining of the mouth, eyes, nose, throat and genitals. This causes blisters and sores that heal with scarring. It mostly affects older adults.
What are the first signs of mucous membrane pemphigoid?
The most common early sign is sore, red, peeling gums or recurring mouth blisters. Some people first notice red, gritty, irritated eyes that do not improve with usual treatments. Nose crusting, hoarseness and painful swallowing can also occur.
Can mucous membrane pemphigoid cause blindness?
Yes. Eye involvement can cause scarring that pulls the eyelids against the eye, turns lashes inward and damages the cornea. Regular eye exams and early immune-suppressing treatment greatly reduce the risk of vision loss.
Is mucous membrane pemphigoid curable?
There is no cure, but it can often be controlled. Some people reach long-term remission and can stop treatment. Treatment aims to stop active blistering and prevent new scarring.
Sources
This guide was written from current patient and clinical guidance, including:
- International Pemphigus and Pemphigoid Foundation: Mucous membrane pemphigoid
- European Academy of Dermatology and Venereology: Guideline on mucous membrane pemphigoid
- American Academy of Ophthalmology EyeWiki: Ocular mucous membrane pemphigoid
- NIH Genetic and Rare Diseases Information Center: Cicatricial pemphigoid
- American Academy of Dermatology: Pemphigoid