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1. Overview

Pemphigus is a rare autoimmune disorder that involves blistering the skin. The immune system produces antibodies with protective role that can be found in the bloodstream, which in turn must protect against hostile attacks of various viruses or bacteria.

In case of pemphigus antibodies mistakenly believe that normal tissues are foreign bodies and attack them. The result is the appearance of painful sores on the skin and mucous membranes (mouth and other moist mucous membranes) that will not cure. In some cases, these lesions can cover a significant area of the skin.


1. Overview
2. Types of pemphigus and treatment modalities

2. Types of pemphigus and treatment modalities

There are several types of pemphigus, each type having specific manifestations and different developments. Still, keep in mind that there are some autoimmune diseases that can present common symptoms (skin blisters) such as bullous lupus and Hailey-Hailey, a condition that can be mistaken for pemphigus.

Since this is a complex disease, special diagnostic investigations are needed. The three main types of pemphigus is paraneoplastic pemphigus, pemphigus vulgaris and pemphigus foliaceus.

The most severe form of pemphigus is paraneoplastic pemphigus. This occurs in people who have already been diagnosed with a type of cancer. The condition is quite rare.

Painful sores in the mouth, lips and esophagus are almost always present. Diagnosis of the disease should be an indication for doctors to look for a hidden tumor.

Pemphigus vulgaris is the most commonly diagnosed form of pemphigus. Wounds and pustules usually appear inside the mouth. Injuries do not always look like bladders or blisters. In pemphigus vulgaris case, there are produced some antibodies in the blood that attack the skin directly.

Antibodies circulate in the blood, then reach the skin and binds to a protein found in skin cells. The function of this protein is normally to maintain skin cells together. If the antibodies bind to proteins, cells disintegrate and blisters appear on the skin and mucous membranes.

When it comes about pemphigus foliaceus, there are developed some scabs, the skin is peeling or fragile blisters occurs primarily on the scalp and then later on the face, chest and back.

Antibodies bind to different proteins of the skin cells compared to pemphigus vulgaris. This protein can be found only on the top layer of dry skin, which is why vesicles are superficial and do not appear in the mouth.

Symptoms that accompany them are itching and mild pain. Pemphigus vulgaris is common among middle-aged adults and elders.

Treatment of first choice in all cases of pemphigus involves taking oral steroids, plus cytotoxic drugs to reduce the side effects of steroids.

Other drugs that may be used are gold injection and cyclosporine. Mild forms of the disease can be controlled with topical or intralesional steroids. Hydroxychloroquine and dapsone were effective in some cases of pemphigus foliaceus.

Medicines can have serious enough side effects so that it will constantly analyze blood and urine. There is some evidence that treatment would be easier in the early stages of the disease. Treatment should always be prescribed depending on the severity of the disease.



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