Overview

The immune system is made up of a complex network of organs, cells, and proteins protecting our bodies. It fights against the invading bacteria, fungi, viruses, parasites and toxins produced by them that protect our bodies.

Our immune system includes antibodies, cytokines, the thymus, spleen, lymph nodes and tonsils, adenoids, and the complement system. 

The immune system keeps a record of every invading organism which gets killed so it can identify the organism as it enters the body again.

A few conditions in which the body organs or cells fail to recognize the outsider microbe or germs and kill its cells. It is known as AUTOIMMUNE CONDITION.

Some of the autoimmune conditions are lupus, type 1 diabetes, rheumatoid arthritis, and myasthenia gravis n. and pemphigus.

What is pemphigus foliaceus?

Pemphigus foliaceus is a rare autoimmune condition. Itchy and painful blisters, fluid-filled cavities, and open sores occur on the skin. The blisters are itchy and crusty rather than painful.

Our immune system produces antibodies which fight microbes and germs. However, the immune system fails to recognize the invader germs and microbes and attacks the skin and mucous membranes of the body.

Who gets affected by Pemphigus foliaceus?

A study shows that both men and women are equally affected by pemphigus foliaceus. It occurs in the age group of 50 to 60 years of age.¹

Which part of the body gets affected by pemphigus foliaceus?

It affects the scalp, face, back, chest, and neck. The blisters occur on the skin and not the mucous membrane in the body.

Symptoms of pemphigus foliaceus

• Superficial blisters form on the skin but do not involve mucous membrane
• Itching of blisters
• Burning sensation
• Localized pain

Management and treatment

The management of Pemphigus varies from person to person and also depends on the severity of the condition. The main goal of treatment is to reduce or suppress the blister formation.³ It was fatal in elderly persons with concurrent medical conditions until the discovery of steroid therapy. The mortality rate has decreased after the advent of steroids and immunosuppressive therapy.

Treatment for mild pemphigus

The first line of treatment includes corticosteroid therapy²

• Corticosteroids clear the blisters and sores in mild pemphigus conditions. Prednisone at 0.5 to 1.5 mg/kg per day or prednisolone 20 to 40 mg per day is advised. A  higher dose of prednisone is given if the symptoms are not under control after two weeks.
• Topical corticosteroids are only for limited or lesser lesions.
• Rituximab, two infusions of 1gm two weeks apart. It is given alone or combined with topical corticosteroids or oral prednisone

The second line of treatment includes

• Rituximab, two infusions of 1gm in fifteen days is given alone or combined with topical corticosteroids or oral prednisone.
• If rituximab is not available or contraindicated, systemic corticosteroids prednisone 0.5-1.0 mg/kg/day with or without azathioprine (1 to 2.5 mg/kg/d) or mycophenolate mofetil 2g/day or mycophenolate sodium 1440 mg/d.

Treatment for moderate and severe types-

• Rituximab  infusions of 1g two weeks apart, associated with systemic corticosteroids (prednisone 1 mg/ kg/day) with a progressive tapering to stop corticosteroids after six months or
• Systemic corticosteroids oral prednisone 1 to 1.5 mg/kg/day alone or associated with an immunosuppressive drug (azathioprine 1 to 2.5 mg/kg/d or mycophenolate mofetil 2g/d or mycophenolate sodium 1440 mg/d.

Additional supportive treatment-

• I am taking care of wounds to prevent them from infection and scarring.
• Use topical creams prescribed by a doctor to control the pain.
• Analgesics like paracetamol, opioids, and metamizole are required.
• Gels containing local anaesthetics are applied on mucosal surfaces for pain relief and soothing effects.
• Mild soaps containing antiseptics such as chlorhexidine should be used by patients with extensive skin lesions followed by moisturizers.
• Protect your skin from sun rays, as ultraviolet rays trigger the blister formation.
• Use sunscreen while stepping out of the home
• Cover the erosive lesions with low-adhesive wound dressings or local emollients and compresses.
• Nutritional management by a dietician or a nutritionist is preferred if malnutrition is related to oral involvement or systemic corticosteroids.

FAQs

How is our body protected from germs or microbes?

The immune system protects our body from germs or microbes. This immune system has complex cells, organs, and proteins which produce antibodies to fight against invaders entering the body.

What is an autoimmune disorder? Few examples

It is a condition in which the cells of the immune system attack its cells as foreign bodies.

Some examples are systemic lupus erythematosus, myasthenia gravis, Sjogren’s syndrome, rheumatoid arthritis, and pemphigus.

What is pemphigus foliaceus?

It is a rare autoimmune disorder with tiny blisters and open sores that are painful on the skin, scalp, face, back and chest.

What causes pemphigus foliaceus?

It can be caused by genetic or environmental factors. The immunoglobulin G (IgG) antibodies are directed against the desmoglein-1, found in the granular layers of the epidermis.⁴

What are the symptoms of pemphigus foliaceus?

Small fluid-filled red bumps or blisters form on the skin. blisters are itchy, scaly, crusty and painful in severe cases.

Pemphigus vulgaris vs pemphigus foliaceus?

In Pemphigus vulgaris the blisters form on mucosal surfaces whereas in Pemphigus foliaceous, blisters and sores form on the skin epidermis.

What is the treatment for pemphigus foliaceus?

Treatment involves steroids and immunosuppressive therapy. Topical corticosteroids are for mild lesions. For moderate to severe lesions systemic corticosteroids, prednisone, rituximab, and dapsone are recommended.

Prevention of pemphigus foliaceus?

Take good care of wounds, apply sunscreen, avoid ultraviolet rays, and use mild soaps and moisturizers on lesions. A nutritious diet is advised.

Summary 

Pemphigus foliaceus is a rare autoimmune disease characterized by blisters and open sores on the skin, face, scalp, back and chest. It is caused by body cells mistaking them as foreign bodies. The blisters are itchy and painful and don't involve mucosa. It is treated by corticosteroids and immunosuppressives.