Bullous Pemphigoid

What is Bullous Pemphigoid?

Bullous pemphigoid is a chronic autoimmune blistering disease of the skin that mostly affects older people. It causes tense blisters on red or normal-looking skin, driven by autoantibodies against two proteins, BP180 and BP230, that hold the skin layers together.^[1] It is the most common autoimmune blistering disease of its type, and it most often appears between ages 60 and 80.^[2]

Common symptoms

Tense blisters and intense, widespread itching are the typical signs.^[2] Itching can come first, and in some people blisters are absent or few, which makes the diagnosis harder.^{[2, 3]}

• Large, firm blisters filled with clear or yellowish fluid, often on the arms, legs, abdomen and groin
• Red, inflamed skin around the blisters, or itchy red patches before they form
• Open sores or crusts where blisters have broken, which can become infected
• A major effect on quality of life^[4]

Causes and risk factors

The immune system makes antibodies that attack the attachment between the outer and deeper skin layers. Genetic susceptibility, environmental triggers and links to other autoimmune disorders are thought to contribute.^[1] Some medicines, skin injury and neurological conditions such as Parkinson's disease have been linked to it, and age is the main risk factor.^{[1, 2]} The number of cases is rising, which is attributed to aging populations and better recognition.^[1]

How it is diagnosed and treated conventionally

Diagnosis combines the clinical picture with a skin biopsy, direct immunofluorescence that shows antibodies at the basement membrane, and blood tests for autoantibodies.^{[1, 2]}

Treatment aims to reduce symptoms and prevent new blisters, using corticosteroids, immunosuppressive drugs and biologics such as rituximab and omalizumab.^[1] A Cochrane review of 14 trials found that clobetasol cream over the whole body probably heals skin as well as oral prednisone, and that doxycycline healed skin less well than prednisolone but was associated with fewer deaths and serious adverse events. Most trials were small, and the evidence for many treatments is very uncertain.^[5] Treating older people who have other illnesses is a particular challenge, and newer drugs aim to reduce the harm from long-term steroids.^{[3, 4]}

Why Bullous Pemphigoid calls for a whole-person approach

Bullous pemphigoid is more than a skin problem. It tends to occur in older adults who already carry a high medical burden, and the disease and its drugs can both affect daily life.^[4]

Specialist treatment controls the immune attack. Supportive care aims at the symptoms and strains around it, such as itch, sleep and stress.

Autoimmune activity

Autoantibodies against hemidesmosome proteins drive blister formation.^[1] This is why treatment relies on drugs that calm the immune system, and complementary care cannot take their place.

Itch and skin comfort

Severe itching is one of the most troubling symptoms, and it can precede the blisters.^[2] Relief of itch is the symptom-level goal most often studied in integrative skin research, as described below.

Aging, other illnesses and treatment safety

BP mostly affects the elderly, who often have other conditions and special safety concerns when treated.^[4] Long-term corticosteroid exposure and conventional immunosuppressants carry adverse effects that newer therapies try to reduce.^[3] Supportive care should be gentle and must fit around this complexity.

Diet and nutrition

A narrative review of autoimmune blistering diseases notes that many patients may need supplements such as vitamin D, and that diets such as the Mediterranean diet and a high-protein, high-calcium diet are used during long-term steroid therapy. It also notes that a soft diet is advised when the mouth is involved.^[6]

Acupuncture as supportive care in Bullous Pemphigoid

What the research shows

There are no clinical trials of acupuncture for bullous pemphigoid. A PubMed search found none, so acupuncture cannot be said to help the disease itself.

Related symptom research is the best available guide, and it does not prove benefit in BP. A 2021 systematic review of acupuncture in skin disease reported improved outcomes in uremic pruritus, atopic dermatitis, urticaria and itch, and called for larger sham-controlled trials.^[7] A 2015 review of 24 studies likewise found improvements for pruritus and some other skin conditions, and asked for better blinded studies.^[8] These studies were mostly in other skin diseases and may not apply to blistering skin.

For chronic pain in general, an individual patient data meta-analysis of 39 trials found acupuncture reduced chronic musculoskeletal, osteoarthritis, headache and shoulder pain more than no acupuncture and, by a smaller amount, more than sham.^[9]

How acupuncture may work

How acupuncture might act in bullous pemphigoid is not known, and no mechanism has been tested in this disease. The pain studies suggest that needling effects cannot be explained by placebo alone, but this does not address blistering or autoimmunity.^[9]

What treatment involves

Acupuncture uses fine, sterile, single-use needles placed at selected points, usually left in for about 20 to 40 minutes. A course of several visits is followed by a reassessment of whether it is helping. With blistering skin, points would be chosen away from blisters, erosions and fragile skin.

Minor reactions such as brief soreness, bleeding or bruising occurred in about 9 in 100 patients in prospective studies, and serious events in about 1 in 10,000.^[10] Tell the practitioner first if you take steroids or other immunosuppressants, which can raise infection risk and make skin more fragile, if you take blood thinners, have a bleeding disorder or a pacemaker, or are pregnant. Report any new blister, redness or fever after a session to your physician.

Ayurvedic medicine as supportive care in Bullous Pemphigoid

The Ayurvedic view

Ayurveda has no classical name for bullous pemphigoid. Practitioners may interpret fluid-filled blisters and itching as an excess of Pitta together with Kapha, with involvement of the blood and skin. This is a traditional framework for choosing supportive care, not a medical diagnosis.

Therapies that may be used

Care is individualized and traditionally may include:

• Diet and daily routine, for example cooling, regular meals and steady sleep and stress-management habits
• Soothing, gentle measures for the skin that your dermatologist agrees with, never applied to open blisters or sores
• Herbs, prescribed one person at a time by a qualified practitioner, with checks against your medicines

Panchakarma cleansing procedures are not appropriate during pregnancy, acute illness, frailty or active flares, or while you are on strong immunosuppressants unless your physician agrees. Because bullous pemphigoid mostly affects older, often frail adults on steroids, we would not use them. Transdermal medication therapy, an herbal preparation applied to the skin, has no research in this disease and would not be applied to broken or blistered skin.

What the research shows

There are no clinical trials or systematic reviews of Ayurvedic medicine for bullous pemphigoid. A PubMed search found none, so we cannot say whether it helps.

Metal contamination is a documented risk with Ayurvedic products. About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[11] Ayurvedic herbs can also interact with prescribed drugs, as other herbs do.^[12]

Herbal medicine as supportive care in Bullous Pemphigoid

Herbs and formulas that have been studied

Research on herbs in bullous pemphigoid is minimal. A review describes traditional Chinese remedies used in autoimmune blistering diseases, but its abstract reports no trial results.^[13] A small retrospective study in China tested Tripterygium wilfordii, a Chinese herb, in 10 patients with mild to moderate BP.^[14] Chinese herbal medicine has also been studied for chronic itch from various causes.^[15]

What the research shows

The evidence for BP is very limited. In the Tripterygium study, 7 of 10 patients had a major response, compared with 9 of 10 given glucocorticoids. Disease control took longer with the herb, relapses occurred during follow-up, and the study was retrospective with only 20 patients in total.^[14] It cannot show that the herb works.

For chronic itch in general, a meta-analysis of 24 randomized trials found Chinese herbal medicine reduced itch and improved quality-of-life scores, with mostly low to moderate certainty, and called for higher-quality studies.^[15] The trials did not specifically cover bullous pemphigoid.

Safety and herb-drug interactions

Herbs can interact with the medicines used for bullous pemphigoid. Reported interactions include St John's wort lowering blood levels of ciclosporin and tacrolimus, and bleeding when warfarin is combined with various herbs.^{[12, 16]} Tell us about steroids, doxycycline, methotrexate, azathioprine, mycophenolate, rituximab and any blood thinner before any herb is considered. A nutrition review also lists St John's wort and Echinacea among herbal supplements reported to worsen autoimmune blistering diseases.^[6]

Some herbs, including Tripterygium, are potent and need expert supervision. Liver or kidney disease, pregnancy and breastfeeding also call for caution. Herbal products can be contaminated, so they should come from tested sources.^[11]

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. Chinese medicine may describe blistering and weeping skin as damp-heat or heat in the blood, and Ayurveda may describe it as aggravated Pitta and Kapha. Researchers instead study autoantibodies against BP180 and BP230 and the inflammation that follows.^[1]

No study has shown that a traditional pattern corresponds to a specific biological process in bullous pemphigoid, and no biological mechanism for acupuncture or herbs has been established in this disease.

How this care fits with your medical care

Bullous pemphigoid needs ongoing care from a physician, usually a dermatologist, with your primary care doctor involved given the age group and common other illnesses.^{[3, 4]} 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.

Do not stop or change steroids, doxycycline, immunosuppressants or biologics without your prescriber. We do not diagnose or manage the disease. Please bring your diagnosis, biopsy and antibody results, and a full list of medicines and supplements.

When to seek urgent medical care

Bullous pemphigoid can cause widespread open skin, infection and serious treatment side effects, so some symptoms need prompt medical help.^{[3, 5]} Get urgent help for:

• Widespread new blisters or large areas of raw skin
• Fever, chills, or spreading redness, warmth, swelling or pus around sores
• Blisters in the mouth, eyes or genitals that make eating, seeing or passing urine difficult
• Blood in stool, black stools or vomiting blood while on steroids or blood thinners
• Confusion, severe weakness or being unable to eat or drink

Call 911 or go to the nearest emergency department for trouble breathing, chest pain, a very high fever with confusion, or any sudden severe symptom.