Pemphigus is a rare auto immune disease of the skin characterised by blister formation on the skin and mucous membranes. Pemphigus is a rare group of blistering autoimmune diseases that mainly affects the skin and mucous membranes of your mouth and of genitals.
Pemphigus disease is characterised by the formation of tense blisters on the skin.
The blistering condition of the skin is caused by abnormal accumulation of antibodies in a layer of the skin called as basement membrane. The epidermal cells of the skin are attached to each other by desmosomes attachment points. At these points, there is present glue like substance known as desmoglein.
In pemphigus auto immune disease, this desmoglein act as an antigen for immune system and antibodies are formed against this antigen. Then these antibodies against desmoglein start accumulating in the basement membrane of the skin and lead to blister formation on the skin.
Blister formation occurs as the auto antibodies attack the desmogleins, the cells start getting separated from each other and hence epidermis is unglued. And this phenomenon is known as acantholysis. This acantholysis causes blisters that slough off and turn into sores. In some cases, these blisters cover a large area of skin depending on the severity of the disease.Classification of pemphigus
Pemphigus is classified into following types:
Pemphigus Vulgaris which is further of many forms:
• Pemphigus vegetans.
• Pemphigus vegetans of Neumann.
• Pemphigus vegetans of Hallopeau.
• IgA pemphigus occurs in many forms
• Intraepidermal neutrophilic IgA dermatosis.
Subcorneal pustular dermatosis.
• Pemphigus foliaceous is of further types given below
Some major types of pemphigus which vary with their severity of symptoms are explained below
• Pemphigus vulgaris:
It is the most common type of pemphigus. In this type, the antibodies attack desmoglein 3. The blisters appear on the mouth and due to which the patient feels difficulty in eating food. This type of pemiphigus occurs at any age but most common at the age of 40-60 years.
• Pemphigus foliaceus:
This type is of least severe type. In this type desmoglein, 1 protein is destroyed by the autoantibody. This type of pemphigus is found in the top dry layer of the skin. There are formations of crusty sores that often begin on the scalp[ and will move to the chest, back and face. In this type, mouth sores are not present.
• Intraepidermal neutrophilic IgA dermatosis:
The characteristic of this type is seen microscopically and characteristics are intraepidermal bullae with neutrophils and eosinophils.
Out of all these types, there is one more type which is severest of all and is known as •
paraneoplastic pemphigus (PNP):
This type is a complication of cancer, usually lymphoma and Castleman’s disease. There are painful sores present on the mouth, lips and the oesophagus. In this type of disease process often involves the lungs, causing bronchiolitis obliterans.
Pemphigus most commonly occurs in older people but the disease can also affect the younger individuals and even babies.
Age of onset of the disease is approximately between 50-60 years of age.
The occurrence of pemphigus in males and females is equal ratio but younger females are more likely to be affected by pemphigus than younger boys.
There is no evidence of any particular race showing more cases of pemphigus.
Pemphigus is an autoimmune disease so its major cause is the production of antibodies against the normal epidermal cells of the skin in mistaken resemblance for viruses and bacteria by the body’s immune response.
Normally immune system of the body attack foreign invaders such as viruses and bacteria.
In some cases, pemphigus may also develop due to side effects of certain medications such as certain blood pressure drugs or chelating agents.
• There are intense itching and burning sensation of the skin.
• In cases where mucous membranes of the skin are affected, then it leads to pain, burning, peeling away of affected inner lining tissues and sensitivity to acidic foods.
• Eating is difficult for the patient and if involvement is in deeper areas of the throat then it leads to coughing.
• If the nose is involved there occurs nosebleed.
• The blisters on the skin rupture easily and leaving open pores.
• In pemphigus foliaceous, the blister begins on the face and scalp and then appear on the chest and back.
• Fever in some cases if blister comes with infection after rupture.
• Achy muscles and joints.
• Examining blisters and on which sites of a body, they are present.
• Skin biopsy at the site of blisters and then examined with the microscope.
• In blood presence of anti-desmoglein auto antibodies.
• Direct immunofluorescence on the skin biopsy also helps to detect antibodies.
The main target of the treatment is to prevent complications
Medications given are as follows
• Biological therapies.
• Antibiotics, antiviral and antifungal medications.
If pemphigus spread is severe then the patient needs to be hospitalised. And the measures to be taken in hospital are as follows
• Fluids are given to maintain the electrolyte balance.
• Intravenous feeding.
• Anaesthetics mouth lozenges are given for mouth pain and soreness.
• Therapeutic plasmapheresis.
• Infection of the skin at the site of blisters.
• The infection spreads through the blood and causes sepsis.
• Medication side effects such as increased risk of infection.
• Very rare death due to severe spread infection.
• Well indicated medicine for pemphigus.
• Bullae on the skin are surrounded by a ring.
• There is severe itching on the skin.
• The face of the patient is very swollen, especially around the eyes.
• There are itching eruptions on the thighs.
• Pustules on the skin.
• Also indicated for uterine cancer.
• Well indicated medicine for secondary eczema.
• Eruptions with mealy scales.
• Vesicular eruptions with burning and itching.
• Medicine also helps in cases of sunburn.
• Burns scalds with rawness and smarting relieved by cold applications followed by undue inflammation.
• There is burning in the soles of feet at night.
• Indicated for intense erythema.
• Vesicles, erysipelas.
• Well indicated medicine for fungoid growth.
• Large blisters, as from scalds.
• There are heavy, brown crusts and scabs on the skin.
• The Very good remedy for the treatment of pemphigus.
• Well indicated medicine for vesicular eruptions, with a tendency to form large blisters.
• Acrid exudations from the eruptions which make the surrounding parts sore.
• Helps in treating pemphigus.
• The skin is red like the flush of scarlatina.
• There is itching and pricking when heated.
• There is pustules and eczema.
• Eczema especially of the lower extremities, sacrum and hands.
• Well indicated medicine for erythema and erysipelatous redness.
• Also, help in treating pemphigus.
• Skin is red, swollen, with intense itching.
• Also indicated for vesicles. Herpes, urticaria, pemphigus, erysipelas and vesicular suppurative forms.
• Also indicated for cellulitis.
• There are burning eczematous eruptions with a tendency to scale formation.