Pemphigus

Pemphigus is an autoimmune disorder that causes painful blisters, bumps and scabs on the surface of the skin similar to eczema and often misdiagnosed as such. It often affects mucous membranes such as the nose, throat, tongue and genitals and can cause blisters in and around the mouth.

In pemphigus, auto-antibodies form against desmoglein which forms the ‘glue’ that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes ‘unglued’, a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a significant area of the skin.

Studies indicate that this condition is quite rare, as it occurs in around only one out of a million people and cases are much higher in the Mediterranean region. There are three types of pemphigus:

Pemphigus foliaceus is the least severe and can be treated quite easily. Desmoglein 1, the protein that is destroyed by the autoantibody, is found in only the top dry layer of the skin. It is characterised by crusty sores that often begin on the scalp and may move to the chest, back and face. Mouth sores do not occur. This form is also frequent among Ashkenazi Jews. It is not as painful as pemphigus vulgaris and is often misdiagnosed as dermatitis or eczema.

Pemphigus vulgaris is the most commonly type and can be quite severe. Sores often originate in the mouth, making eating difficult and uncomfortable. Although it may occur at any age, it is most common among people between the ages of 40 and 60 especially Ashkenazi Jews.

Paraneoplastic pemphigus is the rarest form but most severe form. This disorder is a complication of cancer, usually lymphoma and Castleman’s disease. It may precede the diagnosis of the tumour. Painful sores appear on the mouth, lips, and the oesophagus. In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans (constrictive bronchiolitis). Though much less frequent, it is still found the most in the Ashkenazi Jewish population.

NOTE:  Hailey-Hailey disease, also called familial benign pemphigus, is an inherited (genetic) skin disease, not an autoimmune disease. It is therefore not considered part of the Pemphigus group of diseases.

Definitive diagnosis requires examination of a skin or mucous membrane biopsy by a professional dermatologist or oral pathologist.

Isothiocyanates, phenols, thiols and tannins are known to trigger outbreaks of pemphigus and therefore it is wise to try a two-week elimination of foods containing these compounds to see if the condition improves. If it does, then the foods can be added back into the diet one at a time with a four-day gap to see if that particular food triggers an attack.

Foods to avoid that contain isothiocyanates

NOTE: Extracts from the brown seaweed Ascophyllum nodosum have been found to increase the total isothiocyanate content in broccoli when used as a fertiliser, indicating a potential transfer of isothiocyanates or their precursors from seaweed to other plants.

Foods to avoid that contain phenols

Sources of phenols to avoid in the form of cinnamic acid

• Baked goods
• Beverages
• Confectionery
• Fruit juice
• Ice cream
• Sauces and condiments

Foods to avoid that contain Tannins

NOTE: Tannins are more concentrated in the skins of fruits and nuts especially red and dark coloured fruits. Tannins can inhibit the absorption of vitamin B12 and iron.

Herbs and spices to avoid that contain high levels of tannins

Food and drinks to avoid that may contain artificial tannins

• Berry juice
• Black tea
• Carob beans
• Chocolate
• Cocoa
• Grape juice
• Orange juice
• Processed meats
• Sour cream
• Wood-aged beer, cider, red and white wines or spirits
• Wood smoked meat and fish

Foods to avoid that are high in thiols

It is also important to avoid fried food, oily or greasy food, any form of sugar, caffeinated beverages, alcohol, soft drinks and juices, dairy products, salty food, hot spices, and processed and refined foods. Many processed foods have artificial additives that can contain the triggers for pemphigus.

Drugs that can trigger pemphigus

• Captopul
• Penicillamine
• Ritampicin

Foods that can be consumed during the elimination diet

NOTE: Although the above foods do not contain the elements that can trigger pemphigus, some may cause allergies for susceptible people. Some coconut products are sulphated to keep them white so should be avoided.

Related subjects

• A-Z of ailments
• Allergies
• Asthma
• Phenol intolerance
• Respiratory disorders
• Salicylates sensitivity
• Skin disorders
• Tannins intolerance
• Thiols intolerance