Bullous pemphigoid: causes, symptoms and treatment

Bullous pemphigoid is the most common bullous disease.

The disease mainly affects Elderly people (after 70 years). In fact, the incidence increases with age: 150 to 180 new cases / year / million residents after 80 years. However, we must not ignore the fact that this disease is also defined by its infantile form.

the Women They are more affected than men.

People whose health is unstable are more at risk. In particular, people with neurological or neuropsychiatric diseases (Alzheimer’s disease, Parkinson’s disease, dementia, stroke, etc.).

People with a family history of the disease are at greater risk. Although this condition is not considered an inherited disease, having certain genes will have a (very low) risk of developing the disease in people who carry these genes.

The factors that contribute to the emergence of the disease are:

• a advanced age ;
• the Recently taken some medications Such as spironolactone, psychotropic drugs, gliptin diuretics (against diabetes);
• a Poor general health ;
• affiliate Neurological or nervous diseases (Parkinson’s disease, Alzheimer’s disease, stroke, dementia…).

At first, the disease is usually manifested by eager and one Erythema of certain areas of the skin (red skin). Itching can be severe. These signs can last several weeks or several months. we talk about pre-bubble stage (primary pemphigoid).

Most of the time, blisters (or bubbles) larger than 5 mm with clear contents appearing later. Sometimes they contain blood, especially in patients taking blood thinners. They sit on erythematous, hive-like plaques, most often on the abdomen or roots of the extremities (eg, the armpits and arms of the upper extremities, and the groin and thighs of the lower extremities). The face and neck are mostly preserved. They are rarely found on the external mucous membranes. The number of new bubbles that appear each day is highly variable: it can be less than 10 in some patients or more than 100 in others.

The bubbles last for a few days and give way to erosions or crusts that don’t leave a scar when they disappear.

However, the appearance of blisters is not systemic in all patients (then we talk about form Non-pruritic bullous pemphigoid).

Finally, in rare cases, the disease manifests itself as itching without any skin lesion (sinus itch).

nothing Never spontaneous recovery Bullous pemphigoid, that is, without treatment. The progression of the disease is characterized by the occurrence relapses in about 53% of cases, which often occurs within three months of starting treatment. The one-year mortality rate is high (30%). The risk of death is particularly high during the first 6 months of treatment and is mainly related to the occurrence of infection or cardiovascular complications. Perhaps this is due to the terrain in which the disease occurs, but also to the side effects of corticosteroid treatments.

The disease usually resolves within 1 to 5 years after starting treatment. It can leave unsightly but harmless brown or white marks that eventually disappear. Late relapses, after several years, are always possible.

The main complications of bullous pemphigoid are:

• pest infection.
• Significant loss of water (dehydration) and albumin, very serious in elderly patients.

Diagnosis of bullous pemphigus The first to suspectphysical examinationExamination of the skin and mucous membranes Dermatologist.

To confirm the diagnosis, the doctor will then perform a local anesthesia Two biopsiesThis means removing two small fragments of skin that are sent to the pathology lab:

• One of them is suffering Histological analysis under a standard microscopewhich makes it possible to find out the depth of separation and thus the formation of bubbles;
• The other is analyzed in direct immunofluorescence (IF)To look for autoantibodies anchored in the skin.

a blood test (biological analysis) It may be taken for other complementary tests. They make it possible to study the type and quantity of autoantibodies circulating in the blood.

In the event of an atypical clinical form or suspicion of another type Autoimmune bullous diseaseOther immunological tests may be performed. In fact, bullous pemphigoid should not be confused with another autoimmune disease bullous (mucous pemphigus, linear IgA disease, acquired epidermolysis bullosa, or pemphigus).

In the first intention, the disease is treated by daily application of cortisone cream. In case of resistance or contraindication, systemic medications (immunosuppressants, oral corticosteroids, etc.) may be prescribed.

It is advisable every day to monitor the number of new bubbles, their penetration, sterilization if signs of infection appear, and their drying (in case of oozing).

next one, cortisone cream It should be applied to the affected areas. The applicable product is Clobetasol propionate : 20 to 40 g/day i.e. 2 to 4 tubes in one application all over the body. affiliate Bandages Protectors (non-adhesive) can be put on Topical corticosteroid.

This treatment is long and delicate, and requires a lot of attention from the patient and his entourage. It is recommended that it be performed by a home nurse.

With this treatment, bullous pemphigoid is usually controlled within 2 to 4 weeks, but treatment must be continued after several months to avoid early relapse.

Application a moisturizer Recommended to avoid skin dryness associated with cortisone.

If resistance to topical treatment, other drugs may be prescribed: oral corticosteroid therapy (prednisone) or immunosuppressive drugs (methotrexate, mycophenolate mofetil). In the event of inefficiency or contraindications to the use of these drugs, consideration is given to prescribing other immunomodulatory drugs (rituximab and omalizumab).

Sometimes, the doctor may decide to combine several treatments such as a topical corticosteroid (clobetasol propionate) with an oral immunosuppressant (methotrexate).

Antibiotics (tetracycline, doxycycline, etc.) may also be prescribed.