Definition: What is bullous pemphigus?
Bullous pemphigus is autoimmune bullous dermatitis (a skin disease) most frequent.
Autoimmune bullous dermatoses are a heterogeneous group of rare diseases with a variable prognosis. It is characterized by the appearance of lesions resulting from an autoimmune reaction and leading to the formation of skin blisters or external mucous membranes.
Bullous pemphigus is associated with the production of antibodies against the BP180 and BP230 antigens. mainly affects Subjects over 70 years oldoften suffer from neurological or neuropsychiatric diseases (dementia, stroke, Alzheimer’s disease, Parkinson’s disease, bed rest …).
It usually starts with eager And The appearance of red spots. then, Tense bubbles burst On the skin (usually sparing the face) and occasionally on the external mucous membranes are distinct. These bubbles heal without scarring.
The diagnosis is confirmed by A.S biopsy. Treatment depends primarily on the application Topical corticosteroids in high doses for several months. In the event of treatment resistance or contraindications, the doctor may suggest oral corticosteroids, immunosuppressants, or immunomodulators. This illness can be covered by Social Security 100% (the disorder is off the list).
The prognosis is maintained, with a mortality rate of approximately 30% at 1 year. Poor general condition, aging and associated neurological diseases are the main risk factors for death.
Causes: What causes bullous pemphigoid?
theBullous pemphigoid is a autoimmune disease It is associated with the abnormal production of antibodies directed against the organism. In this case, antibody production is directed against the BP180 (BPAG2) and BP230 (BPAG1) antigens. Alteration of these antigens disturbs the cohesion between the dermis and the epidermis and causes the formation of bullae (or blisters) which then erode before disappearing without leaving scars.
Bullous pemphigoid: who is at risk?
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.
What factors favor bullous pemphigus?
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…).
What are the symptoms of bullous pemphigoid?
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).
What is the development of bullous pemphigoid?
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.
What are the complications of bullous pemphigoid?
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).
What are the treatments for bullous pemphigoid?
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.
Primarily, topical treatment with corticosteroids
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.
Second-line drugs that target immunity
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.