Cicatricial Pemphigoid

What is Cicatricial Pemphigoid?

Cicatricial pemphigoid is a rare, chronic autoimmune blistering disease that can scar. It may affect the skin, the mucous membranes or both. When it is limited to the mucous membranes it is often called mucous membrane pemphigoid, and when it is limited to the eyes it is called ocular pemphigoid.^[1]

In this disease, antibodies attack proteins, most often one called BP180, that hold the surface layer of the mucous membrane to the tissue beneath it.^{[2, 3]}

Common symptoms

The mouth and the eyes are the most common sites. Lesions outside the mouth tend to heal with scarring, which can lead to vision loss and, more rarely, trouble breathing or eating.^[2] Symptoms depend on the site:

• Painful sores, blisters or sore gums in the mouth
• Red, dry, burning or irritated eyes, and scarring of the surface of the eye and lids
• Involvement of the nose, throat, voice box, esophagus or genital area^[3]
• Blisters or sores on the skin in some people

Ocular involvement occurs in about 70% of all people with the disease.^[4]

Causes and risk factors

The exact cause is not fully understood.^[5] The immune system produces autoantibodies against the structures that anchor the mucosal lining, and this leads to inflammation, blistering and scarring.^[3] A family tendency toward autoimmune disease and other triggers have been suggested, but none can be named as a cause in an individual person. The disease is rare, with an incidence of about 2 per million people per year in central Europe.^[2]

How it is diagnosed and treated conventionally

Diagnosis is made by a physician from the pattern of mucosal lesions and a biopsy that shows antibodies stuck to the basement membrane under direct immunofluorescence testing. Blood tests for circulating autoantibodies add information.^[6] Some people with antibodies against laminin 332 have an associated cancer, so their physicians may look for one.^[2]

For mild to moderate disease, European guidelines recommend dapsone, methotrexate or tetracycline antibiotics, and/or topical corticosteroids. For severe disease they recommend dapsone, cyclophosphamide and/or oral corticosteroids. The guidelines note that high-quality randomized trials are lacking.^[6] Corticosteroids and other broad immune suppression remain the mainstay of care and often cause severe side effects.^[5] Eye disease needs close ophthalmology follow-up because of the risk of scarring and vision loss.^[4]

Why Cicatricial Pemphigoid calls for a whole-person approach

The disease itself is driven by the immune system and has to be treated medically. Alongside it, people often live with pain, dryness, trouble eating, poor sleep, stress and the side effects of treatment, and these are the areas where supportive care can reasonably be considered.

Because the disease can affect several organs, we ask that your physicians, which may include a dermatologist, ophthalmologist and dentist or oral medicine specialist, stay in charge of the disease.

Mucosal surfaces and the eye

Scarring of the eye surface can lead to ocular surface failure, infection and loss of vision.^[4] Dry, irritated eyes and sore mouths are the symptoms that most affect daily comfort, and these need your specialists' own eye and mouth care.

Eating and mouth comfort

Mouth involvement, and in some people involvement of the throat and esophagus, can impair food intake.^[2] Pain on eating can affect nutrition and mood, so dietary advice from your physician or a dietitian matters.

Treatment side effects

Corticosteroids and other immune-suppressing drugs often cause severe adverse effects, which is why safer options are being sought.^[5] Side effects are a main reason to coordinate any supportive care with the prescribing physician.

Stress, sleep and pain

Long-term illness can bring stress, worry, broken sleep and pain. These symptoms have been studied with acupuncture and with some herbs, but in other groups of patients, not in cicatricial pemphigoid, as described below.^{[7, 8]}

Acupuncture as supportive care in Cicatricial Pemphigoid

What the research shows

No clinical trials of acupuncture in cicatricial pemphigoid were found in PubMed. The evidence below is for related symptoms in other patient groups, and it cannot be assumed to apply to this disease. Acupuncture does not treat the underlying autoimmune process.

Dry eye is the closest studied symptom. A meta-analysis of 21 trials (1,542 eyes) in typical dry eye, not pemphigoid, found that acupuncture improved tear break-up time, tear production and corneal staining more than artificial tears alone.^[9] A later meta-analysis of 16 trials found that acupuncture added to artificial tears improved these measures but not the eye surface disease symptom score.^[10] Dry eye caused by scarring from pemphigoid is a different problem, and eye specialists must direct its care.

For dry mouth, a review of 10 trials found the quality low and the results inconclusive.^[11]

For chronic pain more generally, an individual-patient meta-analysis of 39 trials in musculoskeletal pain, osteoarthritis, headache and shoulder pain found acupuncture better than sham and than no acupuncture, with the effect persisting at one year with only a small decrease.^[7] Those pains are not mucosal pain. A review of 24 trials in insomnia found that acupuncture improved sleep scores compared with medication, with benefit apparent after about three weeks, although results varied widely between trials.^[8]

How acupuncture may work

A review of laboratory and clinical research proposes that acupuncture acts through the nervous, hormonal and immune systems together. The authors describe this as a hypothesis, and it has not been shown to change the course of pemphigoid.^[12]

What treatment involves

Treatment uses fine, sterile, single-use needles, usually left in place for about 20 to 40 minutes, with a short course of visits and reassessment. In this condition, treatment is aimed at comfort, sleep and stress rather than the disease.

In prospective studies, about 9 in 100 patients had a minor reaction such as pain or bleeding at a needle site, and serious events occurred in about 1 in 10,000 patients.^[13] Tell the practitioner first if you take immune-suppressing medicine, since infection risk matters, and if you have open sores on the skin, a bleeding disorder, take blood thinners, are pregnant, or have a pacemaker.

Ayurvedic medicine as supportive care in Cicatricial Pemphigoid

The Ayurvedic view

Ayurveda has no single classical name for cicatricial pemphigoid. Blistering and ulcerating conditions are traditionally described as involving aggravated Pitta, the principle linked to heat and inflammation, together with disturbed blood tissue (rakta). These are traditional categories, not biomedical diagnoses.

Therapies that may be used

Care is chosen for the individual and, in a supportive role, may include:

• A diet and daily routine traditionally described as cooling and calming, adapted so that nothing irritates sore mouth tissue
• Stress-reducing practices and regular sleep hours
• Herbs such as turmeric or ashwagandha, prescribed individually after review of your medicines

Panchakarma cleansing procedures are not appropriate during pregnancy, acute illness or frailty. They are also generally not suitable during active flares or while taking immune-suppressing medicine unless your physician agrees. Nothing should be put in or near the eyes.

What the research shows

No Ayurvedic trials in cicatricial pemphigoid were found, so there is no evidence of benefit for the disease. Turmeric (curcumin) has been tested in other autoimmune diseases. A meta-analysis of 31 trials across 10 such diseases noted that most trials were small and that the meta-analysis found no efficacy in oral lichen planus. Pemphigoid was not among the diseases covered.^[14]

For stress, a meta-analysis of 9 trials (558 people) found that ashwagandha extracts lowered scores on stress and anxiety scales and blood cortisol compared with placebo. Four trials reported mild to moderate adverse events, and long-term safety is not established.^[15] These trials were not in people with pemphigoid.

Herbal medicine as supportive care in Cicatricial Pemphigoid

Herbs and formulas that have been studied

Chinese herbal medicine has been described for autoimmune blistering disorders. A 2015 review discussed remedies used in pemphigus and bullous pemphigoid, a related but different disease, and called for studies of the active components.^[16] Turmeric and ashwagandha, covered above, are the other herbs with some research on related issues.

Transdermal medication therapy, meaning an herbal preparation applied to the skin, should not be applied to open sores or near the eyes.

What the research shows

No clinical trials of herbal medicine in cicatricial pemphigoid were found. The 2015 review of Chinese remedies for blistering diseases does not report trial results in its abstract.^[16] The evidence for herbs in this disease is therefore none, and only the symptom-level evidence described above exists.

Safety and herb-drug interactions

This is the most important section for this condition, because many people take immune-suppressing drugs. Herbs and prescription drugs can interact, and published cases include St John's wort lowering blood levels of cyclosporin and tacrolimus, with organ rejection reported after cyclosporin levels fell.^[17] Herbs described as immune-stimulating may also work against the purpose of your treatment, so each herb must be reviewed with your prescriber.

Case reports describe bleeding when warfarin was combined with several herbs, including dang gui, dan shen, ginger and Boswellia.^[18] Herbal and dietary supplements account for about 20% of drug-induced liver injury cases in the United States, which matters if you take methotrexate or other drugs that affect the liver.^[19] In one study, about one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[20] Use only tested products prescribed individually, and tell us if you are pregnant or breastfeeding or have liver or kidney disease.

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. Chinese medicine may describe blistering, ulceration and dryness through patterns such as heat or dampness in the channels, depleted yin fluids or stagnant blood. Researchers instead study immune signaling, inflammation and the nervous system's role in regulating both, and some propose acupuncture acts on this network.^[12]

In Ayurveda, aggravated Pitta and disturbed rakta describe heat, redness and ulceration. They might loosely parallel inflammation, but no study has shown that a dosha corresponds to a biological process, or that either framework alters the autoantibody process in pemphigoid.

How this care fits with your medical care

Supportive care here is complementary. Cicatricial pemphigoid needs a physician's care, and we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Do not stop or change prescribed drugs, eye drops or procedures without your prescriber.

We do not diagnose or manage the disease. Please bring your diagnosis, biopsy or antibody results, a full list of medicines and supplements, and the names of your specialists. Report new symptoms to your physicians rather than waiting for a visit with us.

When to seek urgent medical care

Cicatricial pemphigoid can cause vision loss and airway obstruction, so some changes need prompt medical assessment.^[1]

• New or worsening eye pain, redness, light sensitivity or any drop in vision
• Hoarseness, a noisy or difficult breath, or trouble swallowing
• Being unable to eat or drink because of mouth or throat pain
• Fever, spreading redness or pus around sores, which may indicate infection
• Widespread new blistering of the skin
• Bleeding that does not stop

For trouble breathing, sudden vision loss or any emergency, call 911 or go to the nearest emergency department.