Pemphigus vulgaris: symptoms and treatments

Pemphigus vulgaris, although it constitutes a rare disease, It has always been of interest to medicine because of its rarity. Its treatment is long term and, although it is detected on time today, it can still cause death.

As a rare skin disease, too its distribution in the world is variable. According to epidemiological studies, there are countries like Israel where five people out of every one hundred thousand suffer from it in one year, while in England it is only one affected every one hundred thousand inhabitants a year.

In pediatric age the risk of pemphigus vulgaris is minimal. The bulk of cases appear in very old people, and with a tendency to become chronic. Between men and women there are no differences in the number of patients in the world.

Being an autoimmune disease, that is to say that the antibodies themselves attack structures of the organism that they do not recognize, it is not contagious. Who suffers it can not transmit it to others by contact or proximity.

The skin blisters generated by the pemphigus vulgaris are not fatal in themselves, but they cause certain complications that they can eventually lead to death when treatment is not established. Among the most frequent complications we can name:

• Dermal infections
• Sepsis: it is the extension of the infection to the blood
• Malnutrition: due to feeding difficulties when suffering from oral blisters

Causes of pemphigus vulgaris

Pemphigus vulgaris is listed as an autoimmune disease. It is characterized by causing blisters on the skin and mucous membranes of the body.

Pemphigus vulgaris is an autoimmune disease where the body's antibodies attack skin proteins. These antibodies destroy the substances that hold skin cells together. When the cells are separated by the attack, the characteristic blisters of the pathology are formed.

A similar situation occurs in another related disease such as pemphigus blistering. In this case, the antibodies attack the proteins that bind the skin's superficial layer –Epidermis- with the intermediate layer –dermis-, separating them.

In the vulgar pemphigus, on the other hand, the ones that separate are cells that are in the same layer of the skin: in the epidermis, the superficial layer. By separating and leaving a larger space with each other, liquids that express as outward blisters accumulate.

Global studies show the suspicion of a genetic component in the disease. By registering more frequent pathology among certain ethnicities, such as Ashkenazi Jews for example, it is assumed that there are hereditary factors involved.

More rarely, drugs have been detected that may have the adverse effect of administration of pemphigus vulgaris. Among them we can mention:

• Penicillamine
• Angiotensin-converting enzyme inhibitors: such as enalapril
• Nonsteroidal anti-inflammatory drugs: such as acetylsalicylic acid or aspirin

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symptom

Pemphigus vulgaris symptoms are blisters on the skin and mucous membranes of the body. In some cases there may be shedding of skin sheets by rubbing or rubbing.

The blisters burst very easily creating the danger of infection. When bursting, the blisters secrete its contents abroad and leave an open space in the affected skin that favors the entry of bacteria.

The typical location and progression of the lesions is that they begin in the mouth, then in the face and mucous membranes of the genitals, to finally expand to the rest of the body. The patient may report pain, but one characteristic for the diagnosis is that the blisters do not itch.

Anyway, even with the present picture, the diagnosis is difficult for any professional. Therefore, some complementary methods are usually requested such as:

• Skin biopsy
• Blood antibody analysis
• Oral and digestive endoscopy

The doctor may request a skin biopsy to corroborate the diagnosis of pemphigus vulgaris.

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Pemphigus vulgaris treatment

The easiest presentation to treat is the one that comes as an adverse effect of a medication that the patient is consuming. In that case, the mere suspension of the drug is usually sufficient. In a short time the blisters disappear.

For the rest of the clinical presentations of pemphigus vulgaris Medications guided by professionals with some experience are required in this type of pathologies. Among the most used therapeutic options we have:

• Corticosteroids: If the blisters are few it can be used in cream. If there is greater extension, the oral route is preferred with pills such as prednisone. As always when corticosteroids are used, medical follow-up to avoid adverse effects is key.
• Immunosuppressants: Being an autoimmune disease, one option is to use medications that slow down the action of antibodies. Medications such as azathioprine, mycophenolate and cyclophosphamide are part of the list that specialists prescribe.
• Third line: When the disease is aggressive and the previously named drugs do not respond, a third line of attack consists of drugs of greater potency and, therefore, greater possibility of adverse effects, such as dapsone or rituximab.

Who suffers from pemphigus vulgaris should see a doctor immediately. The treatment needs to be instituted quickly to avoid the complications of the disease. Due to its characteristics, it is not a pathology that can be managed without the correct professional advice.