hereditary benign intraepithelial dyskeratosis
Introduction
Introduction to hereditary benign intraepithelial keratosis Hereditary benign intraepithelial dyskeratosis (hereditary benignintraepithelialdyskeratosis) is a congenital syndrome characterized by the appearance of asymptomatic white patches on the oral mucosa and the appearance of blister-like, gelatinous patches on the conjunctival conjunctiva. Autosomal dominant inheritance. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: conjunctival hyperemia
Cause
Hereditary benign intraepithelial keratosis
(1) Causes of the disease
Hereditary benign intraepithelial keratosis is caused by autosomal dominant inheritance.
(two) pathogenesis
Department of autosomal dominant hereditary disease, the pathogenesis is still not very clear, histopathological manifestations of epidermal hyperplasia, acanthosis hypertrophy, vacuole-containing spine cells and benign keratinocytes. The epidermis is mainly waxy eosinophils.
Prevention
Hereditary benign intraepithelial keratosis prevention
Pay attention to eye hygiene, protect your eyes, don't read under strong light, dark room stay time should not be too long, light must be soft enough, don't overuse your eyes. Comprehensive conditioning of systemic complications. Attention should be paid to the effects of drugs and risk factors. Once there are signs of symptoms, they must actively cooperate with the treatment to prevent sudden loss of visual function.
Complication
Hereditary benign intraepithelial keratosis Conjunctival congestion
Damage to the eye of the child can cause permanent blindness with the formation of blood vessels in the cornea.
Symptom
Hereditary benign intraepithelial keratosis symptoms common symptoms visual acuity photophobia conjunctival hyperemia lip folds increased deformity
The damage mainly occurs in the mouth and eyes. The oral cavity mainly consists of buccal mucosa, red lips, under the tongue and gums. The lesions are asymptomatic soft, white sponge-like folds. When the affected mucosa is unfolded, the opaque needle tip is large. The bulge can occur, the damage of the mouth occurs immediately after birth or soon after birth, the damage of the eye has appeared within one year after birth, a small conjunctival macular is formed on the base of conjunctival hyperemia, and photophobia is a common symptom. First, especially in children, permanent blindness can occur with the formation of blood vessels in the cornea.
Examine
Hereditary benign intraepithelial dysplasia
Histopathology: manifested as epidermal hyperplasia, acanthosis hypertrophy, vacuole-containing spine cells and benign keratinocytes, the epidermis is mainly wax-like eosinophils, this cell is called intracellular cells.
Diagnosis
Diagnosis and differentiation of hereditary benign intraepithelial keratosis
According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed. Generally not confused with other diseases.
