What Is Bullous Pemphigoid?

Bullous pemphigoid, also known as senile pemphigus, is a chronic bullous skin disease that occurs in the elderly and is generally considered to be an autoimmune disease. The rate is similar, even in children. The pathology was subepidermal blisters with good prognosis.

Basic Information

nickname

Pemphigoid

English name

Bullous Pemphigoid

Visiting department

dermatology

Disease site

skin

Disease characteristics

On erythema or normal-looking skin, there are large blisteres of cherry to large walnuts, the blister wall is tense, not easy to break, the blister fluid is clear or mixed with blood, etc., with itching

Multiple people

Seniors over 60

Causes of bullous pemphigoid

It is generally considered to be an autoimmune disease. Most patients have anti-basement membrane band autoantibodies, and antigen-antibody binding causes damage to the basement membrane band to form a blister.

Clinical manifestations of bullous pemphigoid

Most patients are over 60 years of age, and the incidence is similar between men and women, and occasionally in children. On erythema or normal-looking skin, large blisteres of cherries to large walnuts appear. The blister walls are tense and not easy to break. The blister fluid is clear or mixed with blood, and the Nissl sign is mostly negative. After the blister was broken, it became erosive and crusted, healed quickly, and pigmentation remained. Occurs in the flexion of the extremities and chest and abdomen. It usually occurs first in one part, and develops into the whole body after half to several months, with itching. About 20% of patients have oral mucosal damage, which is usually mild. According to the scope and shape of the skin lesion, it can be divided into the following types.

Generalized bullous type

This type is the most common.

2. Vesicular type

Clusters of vesicles resemble herpes-like dermatitis.

3. Erythema type

It is predominantly erythema, similar to polymorphic erythema.

4. Pemphigoid

Rarely, the age of onset is usually under 50 years old, with clusters of erythema, pimples, and blistering on the trunk and extremities.

5. Limited bullous pemphigoid (LBP)

About 15% of pemphigoid is common in female lower extremities; it can also be limited to the head and face or upper limbs.

6. Nodular Pemphigoid

The lesions are similar to nodular prurigo, with DIF showing pemphigoid characteristics.

Bullous Pemphigoid Diagnosis

Disease characteristics

Occurs in the elderly, has generalized or limited bullae, is located on erythema or normal skin, the blister wall is tense, and it is easy to heal after breaking.

2. Histopathology

Newly developed damaged tissue can be seen under the epidermal tension blister, the top of the blister is a complete epidermis, the blister fluid contains eosinophils and neutrophils, and inflammatory cells infiltrate the dermal dermis.

3. Immunopathology

Take DIF examination of normal skin near erythema or blister: more than 90% of patients can see continuous linear IgG and C3 deposition in the basement membrane; immunoelectron microscopy shows that these deposits are located in transparent plates under the cytoplasmic membrane of the basal cells; about 70% in IIF Patients had anti-basement membrane band antibodies, mainly IgG, but antibody titers had no parallel relationship with disease activity.

Bullous Pemphigoid Treatment

Glucocorticoid

If the dosage is appropriate, it will be effective in 3 to 5 days. The blisters will dry up and there will be no new damage. The damage will heal in 7 to 14 days and the erythema will disappear. After maintaining this amount, it can be gradually reduced. After several months to several years of treatment, it will gradually decrease. Withdrawal.

2. Immunosuppressive

Such as azathioprine or cyclophosphamide combined with glucocorticoids can reduce the amount of hormones. Patients with a mild condition or contraindications to hormone therapy can also use immunosuppressants alone. It is also reported to be effective with methotrexate (MTX), cyclosporine, or mycophenolate mofetil.

3. Tetracycline and Nicotinamide Therapy

The mechanism is that tetracycline can significantly inhibit the chemotaxis of leukocytes, nicotinamide can stabilize the mast cell membrane, prevent its degranulation, inhibit the release of inflammatory mediators, and inhibit 3,5-cyclophosphoadenosine phosphodiesterase, and also inhibit The release of lysosomal enzymes has a synergistic effect. The side effects of this therapy are small and worthy of application.

4. Other

Sulfadiazine (SP), compound sirolimus, etc. can be used in patients with localized or lighter types. Severe conditions can also be treated with plasma exchange or IVIG.

5. Local treatment

With pemphigoid, local topical glucocorticoid therapy has a better effect.