Autoimmune bullous diseases (AIBDs) are a group of conditions marked by the formation of blisters and erosions on the skin and mucous membranes. Among these, bullous pemphigoid (BP) stands out as the most common, particularly affecting the elderly. This article delves into the treatment approaches for bullous pemphigoid, with a special focus on the role of diet and nutrition in managing this condition.
Understanding Autoimmune Bullous Diseases
Autoimmune bullous diseases (AIBDs) occur when the body’s immune system mistakenly attacks proteins crucial for maintaining skin integrity. This leads to the separation of skin layers and the formation of blisters. AIBDs are classified into different types and subtypes based on clinical, histopathological, and immunological characteristics. While genetic predisposition, medications, infections, hormonal changes, chronic stress, and other autoimmune diseases may contribute to the development of AIBDs, nutrition can also play a significant role.
Bullous Pemphigoid: An Overview
Bullous pemphigoid (BP) is an autoimmune disease where the body produces antibodies that attack the skin, starting an inflammatory reaction, and then causing blisters and lesions on the outer layer of the skin. It typically occurs in people over 60 years of age, with the incidence estimated at 0.2-4 cases/100,000/year. Patients with pemphigoid often suffer from neurological and psychiatric diseases. Sometimes, pemphigoid is paraneoplastic syndrome. Literature data indicate the possible occurrence of cross-immune reactions between skin and central nervous system antigens. The antigens in this autoimmune disease are hemidesmosomal proteins within the dermal-epidermal junctions with a molecular weight of 180 kD (BP180 antigen) and 230 kD (230BP antigen). When autoantibodies combine with antigens, the inflammatory cascade involving neutrophils, eosinophils, and complement components is activated, which leads to damage to the dermal-epidermal junctions.
Symptoms of Bullous Pemphigoid
Bullous Pemphigoid can start with itchiness and the formation of raised bumps in its initial stages. The common symptoms of BP include:
- Itchy skin
- Raised bumps
- Large blisters filled with fluid or pus
- Darkening of the skin surrounding the blisters
- Hive-like rash
- Mouth sores
- Small blisters of the mucous membranes
Blisters may occur anywhere, but often develop on flexural areas of your skin, such as under your armpits (axilla), around your groin or on your stomach (abdomen). In some cases, blisters also form on mucous membranes, including your mouth, tongue, throat, esophagus and/or eyes.
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Diagnosis
Diagnosis of bullous pemphigoid is based on the clinical picture, histopathological examination, and immunopathological test results. Histopathological examination reveals a subepidermal blister and an inflammatory infiltrate with a predominance of eosinophils located around blood vessels. The basis of diagnosis is the detection of linear deposits of IgG and/or complement component C3 along the basement membrane in direct immunopathological examination. Often, indirect immunopathological examination, i.e., of the patient’s serum, detects circulating antibodies against pemphigoid-typical antigens BP180, BP320, or other antigens. The concentration of antibodies does not correlate with the activity or severity of the disease.
Treatment Approaches
The primary goals of bullous pemphigoid treatment are to heal the skin, relieve itching and pain, and prevent new blisters.
Medical Treatments
Corticosteroids: The main treatment involves corticosteroid medicine applied to the affected area, such as a strong steroid cream like clobetasol propionate. Long-term use this type of medicine comes with the risk of skin thinning and easy bruising. A healthcare professional might also suggest a steroid medicine taken by mouth. Systemic glucocorticoids are also effective in the treatment of this disease; however, due to the older age of patients, many comorbidities, and increased exposure to the development of side effects, experts advise against this method of treatment as the first line.
Antibiotics: Medicines that target the immune system.
Immunosuppressants: Some medicines can stop your immune system from attacking healthy tissues. Examples are azathioprine (Azasan, Imuran), rituximab (Rituxan), mycophenolate (CellCept) and methotrexate (Trexall). These medicines also have a risk of harmful side effects, including infection.
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Other Drugs: Other drugs used in the treatment of pemphigoid include methotrexate, tetracyclines, azathioprine, mycophenolate mofetil, cyclophosphamide, and dapsone.
The first-line treatment is strong local glucocorticoids (clobetasol propionate), which are used for a few months after the lesions disappear.
Supportive Care
Wound Care: Following wound care advice is crucial.
Activity Limitation: Limiting activities may be needed. Blisters on the feet and hands can make it difficult to walk or to go about daily tasks. The itching may make it hard to sleep.
Skin Protection: Protecting your skin is essential. Try not to scratch the affected area and wear loose-fitting cotton clothes.
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The Role of Diet and Nutrition
While there is no specific diet that can cure bullous pemphigoid, dietary modifications can play a supportive role in managing the condition. Nutrition is one of several factors that may be linked to the development of AIBDs. The literature mentions various food products and food ingredients acting as disease modifiers.
Identifying Trigger Foods
Some patients report that certain foods trigger or worsen their condition. Common culprits include:
- Thiols: Found in onion vegetables (Allium)
- Phenols: Contained in mangoes and pistachios
- Tannins
- Cinnamic acid
- Walnut antigens
- Nickel: which may be present in a surprisingly large number of food products including black tea, nuts, and seeds, and some canned or processed foods, among many others;
- Milk
- Iodine: which can be found in some meat and fish, iodized salt, and some dairy and eggs;
- Tomato
- Gluten
Keeping a food calendar or journal to track food intake and disease activity can help identify potential triggers.
General Dietary Recommendations
The Bullous Pemphigoid diet plan consists of soft, non-spicy foods that are easy to digest. Choose healthy options that are rich in nutrients to boost your overall health. Substitute herbs instead of chilies and pepper to make the food palatable. Consuming foods in hot conditions may hurt the infected areas. Refrain from drinking citrus fruit juice like orange juice, which will irritate your sores and cause new sores. Fruits are excellent sources of vitamins, minerals, and antioxidants. Eating fruits reduces the inflammatory reaction of the blister formation, provides energy to fight fatigue, and reduces the appetite for sweets and junk foods.
For individuals with oral involvement, a soft diet is recommended. This includes foods that are:
- Soft-cooked
- Mashed
- Blended
- Pureed into smoothies
Examples of suitable foods include:
- Custard
- Puddings
- Applesauce
- Steamed foods
- Mashed vegetables
- Soft-cooked vegetables
- Blended soups
- Oatmeal
It is also advisable to eat small, frequent meals rather than large meals. Consume protein-rich foods such as chicken in its minced or shredded form. Avoid adding too many spices to fish while cooking.
Nutritional Supplementation
Many patients with ABIDs may require supplementation, particularly of vitamin D and B3, calcium, potassium, zinc, selenium, and cobalt.
Vitamin D: People with BP have been found to have lower-than-normal levels of vitamin D. While there is no conclusive evidence that adding Vitamin D can help protect you against a flare of BP, it is important to ask your doctor what's right for you. Vitamin D is essential for Pemphigoid patients as the lack of this vitamin and intake of certain supplements can deplete the absorption of vitamin D.
Nicotinamide: Nicotinamide supplements - a vitamin B3 or niacin- in combination with an anti-inflammatory antibiotic, like tetracycline, have been shown to be an effective treatment for BP. In one study, nicotinamide supplements had the same effectiveness as oral prednisone, but with fewer side effects.
Foods to Avoid
Avoiding acidic fruits and hard foods in the diet helps to prevent irritation of the mouth sores and small blisters.
Other Considerations
Environmental Factors
The common environmental factors known for triggering BP flares are:
- Infections
- Certain medications
- UV light
Hygiene and Skin Care
Proper hygiene and care can help you get rid of the sores and blisters in a shorter duration. Take care of your skin. Clean the blisters with mild soap (neutral pH) and clean water and pat dry the skin with a clean soft towel/cloth. Popping the blisters is not advised until your dermatologist recommends it.
Complications
Bullous Pemphigoid may be a serious condition if untreated. The disease can be fatal in patients with a weak immune system and poor health. The blisters may penetrate deeper into the skin and spread to your bloodstream (sepsis) if the symptoms are ignored.
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