Pemphigus

Symptoms of pemphigus

Treatment of pemphigus

Bubble is accompanied by the appearance of bubbles on healthy skin or mucous membranes, which have a tendency to spread and merge.

It can occur at any age, but people who are 40–60 years old are more likely to suffer, women get sick more often than men.

Symptoms of pemphigus

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Vulgar (ordinary) form – on normal skin or mucous membranes there are single bubbles, sometimes reaching the size of a walnut, with a transparent, gradually turbid, rarely bloody contents. Some bubbles subside, and their contents shrink into crusts, others burst and form ulcerations.

Seborrheic ( erythematous ) pemphigus Senira – Asher begins more often on the face, back, chest or scalp. Bubbles of small size appear, quickly shrinking into grayish-yellowish crusts, upon removal of which they reveal a wound surface.

Pemphigus pemphigus: a disease of the mouth and extremities – a rash on the fingers and toes, palms and soles; often accompanied by the appearance of painful blisters, ulceration of the mucous membrane of the cheeks and tongue, a small fever; the causative agent is Coxsackie virus .

The diagnosis of the pemphigus is carried out by a dermatologist and is based on a set of examination results, cytological, histological and immunological examinations. In all forms of the disease, there is a deposition of immunoglobulin G in the surface layers of the skin.

Treatment of pemphigus

For the treatment used hormones, for example, prednisone inside and to the affected area locally – betamethasone.

Pemphigus is one of the few diseases in which the appointment of hormones is made for health reasons, and the existing contraindications in these cases become relative. Stop taking hormones or reduce their daily dose is recommended very carefully and gradually. Thus, during treatment, as the apparent improvement occurs, the daily dose of the hormone is gradually reduced, about 1 time in 4-5 days, by 2.5-5 mg of prednisolone until the minimum maintenance effective dose of the hormone is achieved, the introduction of which ensures the remission of the disease .

The combined use of cytotoxic drugs, immunosuppressive ( sandimmune ) drugs with hormones allows, in a shorter time and with lower daily doses of hormones, to achieve good results in the treatment of pemphigus.

Azathioprine is used in 2-4 doses per day in combination with hormones.

Methotrexate is used once a week.

Sandimmune for the treatment of patients with true pemphigus is used in combination with hormones, and the daily dose of hormones decreases 3-4 times and corresponds to 25-50 mg of prednisolone. The first 2 days for assessing the tolerability of the drug, Sandimmune is prescribed in half the dose, then the daily dose is divided into 2 doses, in the morning and evening, with an interval of 12 hours. The decrease in the daily dose of Sandimmune begins after the healing of existing ulcerations.

Complete cleansing of the skin should not be considered the ultimate goal of treatment. After achieving remission, the patient should then take the minimum effective maintenance dose of sandimmune , which should be selected individually. In this dosage, the drug can be used for a long time (2-4 months) as maintenance therapy.

For additional therapy of the pemphigus successfully apply methods of blood purification – hemosorption, plasmapheresis.

The effect of hemosorption is explained by the possibility of removing immunoglobulins, circulating immune complexes and other disease-causing components. Hemosorption can be carried out in patients with severe comorbidities (diabetes, hyperthyroidism, atherosclerosis, etc.).

Plasmapheresis – plasma transfusion with an interval of 7-14 days. With each procedure, from 500 to 2000 ml of plasma is removed and fresh donor plasma or plasma substitute is introduced instead . Thus, the circulating immune complexes are removed from the blood of patients.

Photochemotherapy for pemphigus: the essence of the method consists in the inactivation of blood cells using G- methoxypsoralen in combination with ultraviolet irradiation of blood cells and their reintroduction into the bloodstream. The effectiveness of the method is high due to the purification of the blood from toxic substances, immunoglobulins.

For the prevention of electrolyte imbalance, use of potassium, calcium.

Bubble proceeds for a long time, for years, with periods of subsidence and exacerbation.

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