What is it?
Pemphigus are diseases that cause blisters on the skin and, sometimes, on the mucous membranes. They share the characteristic location of bubbles in the most superficial layer of skin, the epidermis.
An autoimmune mechanism produces antibodies against the skin, causing the loss of adhesion between epidermal cells, which forms the blisters.
There are different types of pemphigus. The two main ones are Pemphigus vulgaris and Pemphigus foliaceous. Pemphigus foliaceous has a variety that occurs in Brazil, in the Midwest region and in the states of Minas Gerais, Parana and Sao Paulo, known as Wild Fire.
Characteristics and symptoms
- Pemphigus foliaceous: mostly affects young adults and children living in rural areas, near rivers and in some indian tribes. The disease is characterized by the appearance of superficial blisters, which easily disrupt, leaving eroded skin and forming areas of reddish skin covered by scales and crusts.
The blisters arise at the head, neck and upper trunk and then spread throughout the body, but do not occur in the mucosa. The lesions are painful, with stinging and burning sensations, which originated the name Wild Fire.
- Pemphigus vulgaris: is the most serious variety and appears most often in individuals aged between 30 and 60. In more than half the cases, begins with painful lesions on the oral mucosa. Later, blisters appear on the skin, which converge and break apart leaving eroded areas, similar to burns, deeper than those seen in pemphigus foliaceous.
The lesions are also extremely painful and the ones on oral mucosa causes pain on swallowing and consequent feeding difficulty, which contributes to worsen general conditions of the patient.
Confirmation of the diagnosis of these diseases is made by biopsy, removing a blister for examining under the microscope.
Treatment aims to suppress the self-aggression by blocking antibodies which attack the skin. The main drug used is corticosteroid in high doses. Often the patient must be hospitalized.
Adjuvant drugs are also used to treat secondary infections that may complicate the disease and to control the side effects caused by corticosteroids. General care, such as cleaning of the lesions, hydration and diet of the patient, are also important.
Other immunosuppressive drugs may need to be associated in cases that are resistant to treatment with corticosteroids. The dermatologist is a skilled professional for the treatment of pemphigus.
- Xeroderma pigmentosum