Familial benign pemphigus
Introduction
Introduction to familial benign pemphigus Familial benign pemphigus (familialbenignpemphigus) is also known as Hailey-Hailey disease, which was reported in 1939. It is a dominant hereditary skin disease, and there are cases of no family history. The disease is rare and can be triggered by friction, sun exposure, injury and bacterial infection. Some people think that this disease has some connection with Darry's disease. basic knowledge Sickness ratio: 0.0001% Susceptible population: often occurs during puberty Mode of infection: non-infectious Complications: Eczema
Cause
The cause of familial benign pemphigus
(1) Causes of the disease
Because of the poor keratinization, the disease is considered to be a variation of follicular keratosis. Acantholytic and keratosis can occur in both diseases, and both diseases may occur in the same patient.
(two) pathogenesis
Electron microscopy revealed changes in tension microfilaments and desmosome complexes or interstitial material formation disorders. This potential genetic defect combined with external stimuli such as friction, heat, damage, cold and bacteria and fungi, especially Candida infection, can be induced. The disease.
Prevention
Familial benign pemphigus prevention
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Familial benign pemphigus complications Complications eczema
Eczema, vesicles are widely distributed, and once broken, they are easily infected.
Symptom
Familial benign pemphigus symptoms Common symptoms Itching plaques, crusting, chills, bloating, spleen and kidney yang
Often occurring in adolescence, occurs in the neck, wrist, groin, vulva, perineum, perianal, medial femoral, axillary and other easily rubbing parts, lesions can be limited or generalized, the basic damage is a group of blisters, blister fluid is very clear early It is turbid and turbid. After rupture, it leaves erosion or thick sputum. The damage can be round, oval or multi-ring. Some are similar to eczema, but there are often loose blisters on the periphery. It can form a clear boundary in the groin area. The exudation, sometimes with the odor of the odor, is positive for the vesicles.
Atypical lesions include maculopapular rash, keratotic papules, papillary-like proliferative lesions, and apical keratotic papules in the palm. The patient may have local irritation or itching.
Examine
Examination of familial benign pemphigus
Histopathology: There is obvious thorny release, fissures, villi or bullae on the basal layer, and the irregular combination of epidermal tissue at the nail-forming site forms a so-called collapsed wall-like appearance, and thorny detachment can also be seen between basal cells. In the granular layer, the leukocyte degranulation phenomenon was not observed, and eosinophils were not seen in the vesicles. Sometimes the pathological changes were extremely difficult to distinguish from pemphigus vulgaris, but the latter had a small range of spine release and no keratinocytes were found. It is not difficult to identify with keratosis of hair follicles. The latter has small basal layer fissures, no blister formation, no significant thorny lysis, and obvious keratinized cells. Cell smears showed incomplete acantholytic cells and dyskeratocytes.
Diagnosis
Diagnosis and identification of familial benign pemphigus
Diagnostic criteria
1. According to clinical blister erosion, exudation, scarring damage occurs in the skin wrinkles, with a family history, combined with histopathological diagnosis is easier to make.
2. TCM syndrome differentiation
(1) Damp heat fumigation type:
Main card: rash with erythema, blisters and erosion, increased in summer, rash in autumn and winter significantly reduced or subsided; with thirst do not want to drink, waist and abdomen full; tongue red, white or yellow moss, pulse string slippery.
Syndrome: hot and humid fumigation, on the skin.
(2) spleen and kidney yang deficiency type:
Main card: prolonged course, erythema, blister is not obvious, mainly with moist proliferative lesions; with cold and cold limbs, abdominal distension and loose stools; pale tongue, white fur, slow pulse.
Dialectical syndrome: spleen and kidney yang deficiency, moisture does not change.
Differential diagnosis
Should be identified with impetigo, body lice, eczema, rash, and proliferative pemphigus, can be distinguished by histopathology and immunological examination, follicular keratosis (Darière disease) histology Similar to this disease, but the clinically predilection site and follicular keratotic papules are significantly different from the disease as the primary rash.
