Epidermolysis bullosa introduction
Epidermolysis bullosa statistics
The area where the layers meet is called the basement membrane. Handle your child gently. Clothing: In younger children, diapers may require additional padding at the legs and waist. Blistering may be localized to the hands, feet, elbows and knees or it may be generalized. For children with mild forms of epidermolysis bullosa, they can protect their skin by wearing long pants and sleeves for outdoor activities. Dress your child in soft clothes. Encourage your child to be active. If an individual cannot swallow easily, they may develop malnutrition. Junctional epidermolysis bullosa JEB is the most severe form.People with DEB have a higher risk of developing squamous cell carcinomawhich is an aggressive form of skin cancer, before the age of 35 years. Patients should avoid prolonged exposure to ambient heat and humidity. Each type has several subtypes.
If blistering in the esophagus makes eating difficult, the doctor may recommend surgery to widen the esophagus. They typically include: blistering on the skin, scalp, and around the eyes and nose tearing of the skin skin that looks very thin skin that falls off milia, or very small, white bumps on the skin loss of fingernails, toenails, or both, or deformity of the nails blisters or erosions of the eye If EB occurs in the mucous membranes, it can cause: difficulties with swallowing, if blistering occurs around the mouth and throat a hoarse voice, due to blistering in the throat breathing problems, because of blistering in the upper airway painful urination that results from blistering in the urinary tract Symptoms typically develop very early in a person's life, often soon after birth.
Later in life, skin on the palms of the hands and soles of the feet may thicken and harden hyperkeratosis. There are rare recessive forms. The doctor may prescribe a topical antibiotic for an infection. In Kindler syndrome, a rare subtype of EB, blisters form at birth.
Epidermolysis bullosa symptoms
Causes Faulty genes cause EB. Junctional epidermolysis bullosa. Oral cavity involvement and irregular pitting of the surfaces of the teeth is common in all subtypes. In Kindler syndrome, a rare subtype of EB, blisters form at birth. Adolescents and adults with certain types of epidermolysis bullosa are at high risk of developing a type of skin cancer known as squamous cell carcinoma. The signs and symptoms of this condition vary widely among affected individuals. Complications Complications of epidermolysis bullosa may include: Infection. This type is related to a flaw in the gene that helps produce a type of collagen that provides strength to the pig-skinlike dermis layer of the skin. It's the most common and least severe type. To protect the baby from further discomfort, pick it up by placing one hand under the child's buttocks and the other behind its back. Blisters in the mouth can make eating difficult and lead to malnutrition and anemia such as low iron levels in the blood. Covering the wound with skin may help the healing process.Keeping the skin cool: Nothing hot should ever be applied to the skin of a patient with EB. Description Epidermolysis bullosa simplex is one of a group of genetic conditions called epidermolysis bullosa that cause the skin to be very fragile and to blister easily. The doctor may teach people how to break any large blisters safely and hygienically.
Other patients have selenium and carnitine or vitamin D deficiencies which may predispose them to cardiomyopathy and osteoporosis. This form of the disorder also involves skin blistering from early infancy, hyperkeratosis of the palms and soles, and abnormal nail growth.
Epidermolysis bullosa simplex is one of the major forms of epidermolysis bullosa.