juvenile pemphigoid
Introduction
Introduction to juvenile pemphigus Juvenile pemphigoid is a chronic skin disease characterized by subepidermal blistering. basic knowledge The proportion of illness: 0.003% Susceptible people: children Mode of infection: non-infectious Complications: sepsis
Cause
Juvenile pemphigoid cause
(1) Causes of the disease
Pemphigus is an autoimmune skin disease, the antibody is mainly Ig, a few are IgA, IgM, 80% of the children can detect the antibody in the serum, the antigen binds to the antibody, and the basement membrane damage is caused by the classical complement activation pathway. Forms clinical skin damage.
(two) pathogenesis
Epidermis, vesicles are mainly eosinophils, and neutrophils, blister epidermis is generally normal, dermal papilla edema, a large number of eosinophils and lymphocytes infiltrated around the superficial vascular filaments and the nipple. There are a few neutrophils.
Prevention
Juvenile pemphigoid prevention
1. Do a good job of cleaning the skin and mucous membranes of infants and young children to prevent infection or injury.
2. Actively look for predisposing factors and pay attention to avoiding them in the future.
3. Pay attention to nutrition, enhance physical fitness, and improve living environment.
Complication
Juvenile pemphigoid complications Complications sepsis
Can be complicated by skin infections, severe cases can occur sepsis.
Symptom
Juvenile pemphigus symptoms Common symptoms Mucosal damage Itching scarring Skin blisters, posterior form... Blister or bullous damage
According to skin damage, it can be divided into bullous type, vesicular type, erythema type and local type. Children are mainly blisters and bullae type. The basic damage is tension blisters and bullae with a diameter of about 0.5cm. The maximum diameter can reach 7cm, the blister wall is thick and tense, the Nissl sign is negative, the blister fluid is clear, even bloody, occurs on the basis of normal skin or erythema, occurs on the trunk and extremities, about 25% of patients have mucosal damage, can be in the mouth Blisters and erosions occur in mucous membranes such as pharynx, vulva and perianal, consciously varying degrees of itching, and if there is no secondary infection after blister rupture, it can quickly dry and crusted, leaving pigmentation or hypopigmentation after sputum detachment Spots, if not properly treated, rashes can appear one after another or in batches.
Examine
Examination of juvenile pemphigoid
30% to 50% of patients have elevated eosinophil counts in peripheral blood, and direct immunofluorescence is performed on the skin around the bullae. 80% of patients have linear deposition of IgG in the basement membrane, and C3 is linear here in 100% of patients. Deposition, a small number of patients can have both IgA and IgM deposition, taking the blood of active patients for indirect immunofluorescence, 70% to 80% of the cases have anti-epidermal basement membrane antibodies in the serum, immunoelectron microscopy shows IgG and C3 deposition On the upper part of the transparent zone of the basement membrane and the hemides of the cytoplasmic membrane of the basal cell, electron microscopy showed that the epidermal dermis separated the transparent zone of the basement membrane.
If clinical symptoms are associated with poisoning, X-ray films, electrocardiograms, etc. should be performed.
Diagnosis
Diagnosis and identification of juvenile pemphigus
diagnosis
According to the characteristics of vesicles, mild to moderate itching, combined with histopathological findings and immunofluorescence, is not difficult to diagnose.
Differential diagnosis
1. Congenital bullous epidermolysis: This disease is more than after birth or within 1 year of age. The blisters are mostly loose blisters, which occur in the friction site. The histopathology is found in the epidermis or epidermis. Infiltration is rare, both direct and indirect immunofluorescence are negative.
2. Herpes-like dermatitis.
3. Children's linear IgA bullous skin disease: The key to the identification of this disease and these two skin diseases lies in the direct immunofluorescence of the skin.
Direct immunofluorescence: Pemphigus-like IgG and C3 are linearly deposited in the basement membrane zone; herpes-like dermatitis is IgA and C3 in the dermal papilla top and/or basement membrane zone in granular deposition, while linear IgA is large The blister skin disease is a bullous skin disease with a homogeneous linear deposition of the IgA basement membrane zone.
