Lectures of Duhring Brocq's disease

2021-03-24 12:00 AM

By direct immunofluorescence test, granular IgA deposition in 85, 90% of patients and sugar formation in Duhring Brocq patients.

Define

Duhring - Brocq disease is a chronic skin disease, available of all ages, classified as an autoimmune disease with 4 typical clinical symptoms, generally good treatment results but relapse prevention. many difficulties.

The disease has a good prognosis in young people, progressing in waves, erratically, the lesions leave dark in colour and then gradually disappear. When the patient is over 60 years old, the prognosis is more conservative or fever, thinning, insomnia and even death.

Pathological aetiology

Up to now, most authors say:

Genetic factors

In DH patients there is a high incidence of 85-90% HLA- B8 and HLA- DRW3. 2.2. Antibody and complement factors.

In 1967 Cormane, Meer (1969) discovered the deposition of IgA antibodies at the papillary apex, in Duhring-Brocq patient, while with bullous pemphigoid, IgG was the most common class of antibodies. 

By direct immunofluorescence test, granular IgA deposition in 85-90% of patients and sugars (streaks at 10-15%) in Duhring-Brocq patients, along with C3 deposition arranged in individual seeds in the dermis.

Associated with autoimmune diseases

Duhring - Brocq disease has been described with a number of autoimmune diseases such as glomerulonephritis, Bermeer anemia, thyroiditis, systemic lupus erythematosus, atrophic chronic polymyalgia.

The role of gluten

Gluten is mentioned in the aetiology of the disease and is considered a common antigen in Duhring-Brocq patients. Gluten is a protein found in grains (excluding rice and corn). Gliadin is included in the composition of gluten. Gliadin binds to reticulin. Reticulin itself is an important component in the basal membrane region, it plays a role in increasing the adhesion of the basilar membrane. Gliadin combines with extracellular substances to increase tissue viscosity. In Duhring - Brocq, an increase in extracellular viscosity is associated with the diffusion of the epidermal tissue and leads to the formation of blisters.

Duhring - Brocq and coeliac disease (CD)

In 1966 Marks, was the first to describe the asymptomatic gastrointestinal disease in Duhring-Brocq patient. Several other authors have also found that small intestinal villi atrophy is secondary to gluten sensitivity and cannot be distinguished from CD disease. Now well defined, both Duhring-Brocq and CD's have gluten-sensitive disorders, have a family predisposition and both have dysfunction in the small intestine. But small bowel disease in Duhring-Brocq patients is usually milder than in CD disease.

Clinical symptoms

Lesions usually appear slowly on a body with almost no special changes, the patient has a mild fever or no fever, fatigue, weight loss, and negligible loss of appetite. In areas of the skin that are about to be damaged, there is often a sign of warning, typically itching, followed by burning or pain.

Lesions are mainly on the skin, symmetrical, on the folds of the legs, forearms, arms, followed by the buttocks, the buttocks, thighs, then the back and abdomen, rarely lesions in the armpit, in the sacral region. Damage to the oral mucosa was detected only in 4.6% of Duhring - Brocq patients.

Lesions at the onset are erythema, blisters, papules, which then gradually become blisters, arranged sporadically or clustered as in ecopathy. These are the size of a stretch of corn, containing a lemon-yellow fluid, and rarely hemorrhagic. Blisters last 5-7 days, then pus and break, leaving slips, crusting, pus, often negative Nikolsky skin signs. Lesions here have many ages, some are erythema, some are blisters, some ulcers, some even leave a dark inlaid. Although there is a purulent burn, but rarely complications of lymphadenitis or lymphadenitis. 

The disease progresses in waves, sometimes increases and decreases, sometimes temporarily stabilizes, but then relapses, there are cases that last a lifetime but the patient is still working almost normally, except for a few cases. In the elderly, long term can be depleted.

Diagnostic tests, differential diagnosis of Duhring - Brocq's disease

Blood tests

There may be an increase in eosinophils> 10% (even 20-90%).

Test ki

The test is performed with oral potassium iodide (KI), a single dose of 1 gram, or by applying topically 50% KI in vaseline to normal skin. Reactions appear 24 to 48 hours in the form of an itchy erythematous rash or itchy blisters.

Tzanck cells

These are Malpighi spine cells with monstrous images due to dissociation by loss of connective bridges. 

Direct immunofluorescence test

This is the most valuable test in the diagnosis of Duhring - Brocq's disease. The positive rate is 85- 90% of cases. The test showed that there was a deposition of IgA in the papillary tip.

Histopathology

The typical picture is blistered below the epidermis. There is no phenomenon of pepper spikes. Lime yellow hydrating fluid. Neutrophil infiltrates and eosinophils predominate. In the dermis, there is an infiltration around the blood vessels by lymphocytes, neutrophils, eosinophils. Image of microscopic abscess at the papillary apex.

Implementing the quadrants

Clinical manifestations (typically with 4 symptoms): pruritus in the skin before lesions, erythema, papules, blisters, blisters. Mucosal damage is very rare.

Nikolsky signs are usually negative.

The disease progresses many times, the whole situation less changes.

Subclinical: eosinophils increase in the blood, positive KI test. Tzack cells are negative. Histopathology is most certain. The direct immunofluorescence test is positive even during stable illness.

Differential diagnosis

Conventional Pemphigus: here the lesion is monomorphic: bullous in the epidermis and negative KI test ...

Bullous pemphigoid: a common disease in the elderly, difficult to rupture, if ruptured, it will heal quickly, immunofluorescence test directly detects the deposition of IgG antibodies and complement in the basal membrane ...

Erythema multiforme: infected with cells as seen in Duhring - Brocq.

Immediately: bullae on the red inflamed skin, quickly burst, leaving a lemon yellow scaly ...

Urticaria: lesions are erythematous, papular, edematous, often itchy, but especially rapid and disappear quickly.

Also differentiated from shingles, bullous itchy papules, congenital bullous epithelium.

Treatment and prevention

In place

Mainly using Milian antiseptic solution, methyl purple, methylene blue to apply to the lesions (if the lesion is still scalded water should use a sterile needle to absorb the fluid before applying). When the lesion is dry, it is possible to apply grease chloride, flucina. 

Body

Corticoid 30-40 mg/day, then reduce the dose gradually in 4-8 weeks, only for severe cases.

Erythromyxin 1 - 1.5 grams / day for 7 days x several sessions.

DDS 50-300 mg daily for 1-2 months.

Sulfapiridin 1 - 3 grams daily, 1-2 consecutive months.

Special attention: motivate the patient's ideology, supplement nutrition, take care at the spot, keep oral hygiene, improve the patient's general well-being.

Prevention: The diet should limit the intake of foods high in gluten such as rice and corn.