Pemphigus

Pemphigus is a rare autoimmune blistering skin disorder characterized by the sores and blisters that develop in the skin or the mucous membranes such as the mouth or the genitals. Pemphigus can develop at any age group but is most often detected among middle-aged or older people. It generally tends to be a chronic condition and some types can also be life-threatening without treatment. The exact reason why this condition develops is not known although research indicates that individuals may be genetically predisposed to it. Also, various triggering factors are believed to trigger or aggravate the condition such as ultraviolet light, certain foods, various drugs and stress.  There are two main types of pemphigus; pemphigus vulgaris and pemphigus foliaceus with each type having subtypes.

Pemphigus Vulgaris

This is the most common type of pemphigus which affects adults between the ages of 30 and 60. In pemphigus vulgaris, the lesions can develop in the mucous membranes which are the thin, moist covering of the body’s inner surfaces. The affected individuals often develop irregularly-shaped painful lesions of the mucous membranes lining of the oral cavity. The blisters rarely can also develop because they rupture immediately resulting in multiple ulcerated sores throughout the mouth. As these lesions are often slow to heal, the affected individuals have difficulty with eating and drinking.

Pemphigus foliaceus

This is characterized by multiple small blisters or crusted lesions that form itchy and scaly lesions. They can also be painful or associated with a burning sensation. The most affected part of the body is the face, chest, back and does not involve the mucous membranes. In some cases, these lesions can disappear on their own without the need for treatment. However, in many cases, the lesions can increase in numbers over several months and in severe cases, it can resemble exfoliative erythroderma which is a condition characterized by the red, scaly lesions covering large portions of the body.

Diagnosis

The diagnosis is based on various tests including blood tests for antibodies and a skin biopsy that reveals the typical histological changes. Also, direct immunofluorescence on a skin biopsy sample may be conducted which enables the antibodies to be identified under the microscope. Detection of specific antibodies confirms the condition and also distinguishes the different forms of pemphigus. In the case of pemphigus vulgaris, an endoscopy may be necessary to detect the sores in the throat.

Standard Therapies

The treatment is based on suppressing the lesions and to prevent the development of complications. Although there is no cure for the disease, the treatment often results in most patients entering a complete remission and going off all therapy. The general treatment for pemphigus is corticosteroids such as prednisone that can also suppress the immune system. Steroids may also be applied to the affected areas and the topical therapy can help reduce pain and to prevent infection. Recently, the FDA approved the use of rituximab as first-line therapy for pemphigus and to prevent the new autoantibodies from developing. Other forms of treatment include the use of immunosuppressive drugs, medications to regulate the immune system (immunomodulatory drugs) or antibiotics.

References

https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409

https://www.aad.org/public/diseases/painful-skin-joints/pemphigus

http://www.pemphigus.org/living-with-pemphigus-pemphigoid/understanding-pemphigus-pemphigoid/

https://rarediseases.org/rare-diseases/pemphigus/