Bullous epidermolysis
Epidermolysis bullosa (EB) is a rare genetic disease that manifests itself as very fragile skin and repeated blisters due to daily minor friction. The disease is not contagious. About one in 50,000 children will develop EB. All races suffer from EB, and the ratio is male to female. EB is divided into three major types: simple, malnourished, and borderline. These types differ in the depth of the blister in the skin. 1. Simplex: the dominant inheritance. The skin lesions were bullae and blisters of various sizes, no signs of spinous layer loosening, no scarring after healing, and temporary pigmentation. The affected children developed normally. Hair, nails, teeth, and mucous membranes are rarely affected. Can be improved to puberty. 2. Dominant dysplasia type: The damage is a loose bullae, the spinous layer loosening sign is positive, and atrophic scars are left after healing, often accompanied by miliform rash and pigmented disorder. Physical and mental development is normal. Hair and teeth are often not affected. Sometimes there are ichthyosis, keratosis around the hair, sweating, or thick nails. 3. Recessive dysplasia type: In addition to the slack blisters, there are often blood blistering, and the spinous layer loosening sign is positive, leaving atrophic scars and pigment disorders after healing, and the mucosa is easily affected. Depending on the site of the invasion, there may be signs of aphonia, difficulty swallowing, and contracture of the gingival. There are dysplasia of nails and teeth, hair loss and deformities such as dwarf, claw-hand, and pseudo-fingers. Cancerous. 4. It can be diagnosed according to the symptoms after birth and the characteristics of blisters. Pediatric pemphigus, pemphigus vulgaris, impetigo, etc. should be identified. Congenital bullous epidermolysis is a group of hereditary skin diseases characterized by the appearance of vesicular bullae on the skin.
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