Pemphigus

What is Pemphigus?

Pemphigus is a group of rare and severe autoimmune blistering diseases caused by antibodies directed mainly against desmoglein 1 and desmoglein 3, proteins that help skin cells stick together. The antibodies make neighboring skin cells separate, forming blisters and erosions.^{[1, 2]}

Common symptoms

The main symptoms are painful, fragile blisters that break open and leave raw erosions. Pemphigus vulgaris, the most common type, often begins with painful sores in the mouth and can also affect the skin; pemphigus foliaceus affects only the skin.^{[1, 3]}

Mouth lesions can make eating and drinking painful. Oral lesions are often a first sign and tend to be harder to treat than skin lesions.^[4]

Causes and risk factors

Pemphigus is an autoimmune disease in which IgG antibodies attack desmogleins.^[2] Paraneoplastic pemphigus is a rare, severe form almost always linked to an underlying tumor, and it can cause widespread mouth and skin damage with a high risk of dehydration and infection.^[5] IgA pemphigus is another rare variant.^[6]

Researchers also describe genetic susceptibility and medicine or infection triggers in some people, but the cause is not fully understood.^[2]

How it is diagnosed and treated conventionally

Diagnosis is made by physicians. It rests on a biopsy next to a lesion showing antibody deposits by direct immunofluorescence, along with blood tests for antibodies against desmogleins.^[1]

Corticosteroids have long been the mainstay of treatment, and the antibody drug rituximab is now widely used in moderate and severe disease.^[1] In a trial of 135 people with pemphigus vulgaris, 40% of those given rituximab were in sustained complete remission at one year, compared with 10% on mycophenolate, and they needed less steroid. Serious adverse events were more common with rituximab.^[7] Other immune-suppressing medicines, intravenous immunoglobulin and plasma exchange are also used.^[2]

Pemphigus is serious and treatment can have significant side effects, so it is managed by dermatologists, often with other specialists.

Why Pemphigus calls for a whole-person approach

Pemphigus is driven by the immune system, so treatment centers on calming it, usually with strong medicines. Living with pain, skin care and long-term medicines can also affect eating, sleep and mood.

Supportive care aims at these day-to-day burdens, not at the antibodies or blisters themselves.

The immune system

Pemphigus is mediated by pathogenic autoantibodies, which is why steroids and B-cell-depleting drugs are used.^[1] Because patients are often on immune-suppressing treatment, infections and drug interactions need extra attention.

The mouth and nutrition

Painful oral erosions can make eating hard, and extensive skin loss can cause fluid and protein loss in severe disease.^{[4, 5]}

Medicine burden and quality of life

Reducing steroid exposure is a goal of modern treatment, and rituximab roughly halved steroid-related adverse events in one review.^[1] In the rituximab trial, quality-of-life scores improved in both treatment arms.^[7]

Sleep and stress

Chronic illness, pain and steroids can disrupt sleep and raise stress. Studies of acupuncture for insomnia and of ashwagandha for stress were not done in people with pemphigus.^{[8, 9]}

Acupuncture as supportive care in Pemphigus

What the research shows

We found no clinical trials of acupuncture for pemphigus, and no evidence that it affects the disease. Anything said here concerns related symptoms in other groups of people.

For sleep, a review of 24 trials in insomnia included a meta-analysis of 15 trials that found acupuncture improved sleep quality scores compared with medication, with clearer effects after three or more weeks.^[8] The trials were not in pemphigus, and the review's reported heterogeneity was high.

How acupuncture may work

No mechanism has been studied in pemphigus. General proposals about effects on stress and sleep pathways are not established for this disease.

What treatment involves

Skin fragility is a real concern, so acupuncture must be adapted to the person and the state of their skin. Needles should not be placed into or near blistered, eroded or fragile skin, and sites and techniques need to be chosen carefully, often avoiding active areas. Sessions typically last about 20 to 40 minutes, and we reassess after a short course of visits.

In prospective studies, about 9 in 100 patients had a minor reaction such as soreness or bleeding at a needle site, and serious events occurred in about 1 in 10,000 patients.^[10] Because immune-suppressing medicines raise infection risk, please tell us about all your medicines. Also tell the practitioner first if you have a bleeding disorder, take blood thinners, are pregnant, or have a pacemaker (relevant to electroacupuncture).

Ayurvedic medicine as supportive care in Pemphigus

The Ayurvedic view

Ayurveda describes blistering skin diseases as a group often called Visarpa or Pitta-predominant skin disorders, involving aggravated Pitta and Rakta (blood). This is a traditional framework, not a biomedical explanation of an autoantibody disease.

Therapies that may be used

Because skin and mouth are fragile, care is gentle and individualized:

• Dietary guidance, such as bland, non-irritating foods when the mouth is sore
• Daily routines for sleep and stress
• Breathing and relaxation practices
• Herbs traditionally used for skin or digestion, prescribed individually

Panchakarma, strong massage and heat are not appropriate with active blistering or erosions, during acute illness, pregnancy or frailty, or on high-dose steroids or immune-suppressing treatment, and we would not use them here.

What the research shows

We found no studies of Ayurvedic treatment for pemphigus. For stress, a meta-analysis of 12 trials found ashwagandha extract reduced anxiety and stress scores compared with placebo, but certainty was low and results varied widely; those trials did not involve pemphigus.^[9]

How ashwagandha interacts with steroids and immune-suppressing drugs has not been well studied, so we would not use it without your dermatologist's agreement. About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[11]

Herbal medicine as supportive care in Pemphigus

Herbs and formulas that have been studied

Chinese herbal medicine has been studied in pemphigus as an add-on to corticosteroids. Tripterygium wilfordii (thunder god vine) polyglycosides are used in Chinese dermatology for autoimmune skin diseases including pemphigus.^[12]

What the research shows

The evidence is weak. A systematic review found four trials with 199 patients and reported that adding Chinese medicine to steroids seemed to help lesion healing and prevent relapse and complications. The trials were not of high quality, only one reported side effects, and safety was uncertain.^[13] A Cochrane review that included one Chinese medicine trial among 11 trials of pemphigus treatments concluded that results for most interventions were inconclusive.^[14]

These findings do not show that herbs treat pemphigus. They do not replace rituximab, steroids or other medical treatment.

Safety and herb-drug interactions

Herbs can be risky with the medicines used in pemphigus. The authors of a network pharmacology study note potential liver toxicity from long-term use of Tripterygium.^[12] Herbs can change levels of immune-suppressing drugs, and St. John's wort lowered ciclosporin and tacrolimus levels, in one case leading to organ rejection.^[15]

Products can also be contaminated, as the lead, mercury and arsenic findings show.^[11] Tell us if you are pregnant or breastfeeding, or have liver or kidney disease. We would not use herbs that affect immunity or liver function without your physicians' agreement.

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. Chinese medicine often describes blistering skin disease as damp-heat or toxic heat in the skin, and Ayurveda as aggravated Pitta and blood. Researchers instead describe autoantibodies against desmogleins, loss of cell adhesion and the roles of T and B cells.^[2]

Some Chinese herbal research proposes that certain herbs act on inflammatory signaling such as IL-17 and Th17 cells, but that work is laboratory and computer-based.^[12] No study shows that a traditional pattern corresponds to a specific immune process in pemphigus.

How this care fits with your medical care

For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Pemphigus needs a dermatologist's diagnosis and ongoing treatment.^[1] Do not stop, delay or lower steroids, rituximab or other prescribed medicines because of acupuncture, Ayurveda or herbs.

Please bring your diagnosis, biopsy and antibody reports, a full list of medicines and supplements, and your dermatologist's contact details. We do not diagnose, monitor or treat the disease itself.

When to seek urgent medical care

Pemphigus is potentially life-threatening, and some changes need prompt medical attention.^[1]

• New or spreading blisters, or large areas of skin peeling
• Fever, chills, or a blister or sore that looks infected, with spreading redness, pus or increasing pain
• Mouth sores that stop you from eating or drinking, or signs of dehydration
• Trouble swallowing or breathing, or eye pain or vision changes
• Weight loss with persistent blistering, which may suggest an underlying cause
• Signs of serious infection, or confusion, while on immune-suppressing medicines

If any of these occur, call 911 or go to the nearest emergency department.