The topic Generalized Severe JEB you are seeking is a synonym, or alternative name, or is closely related to the medical condition Junctional Epidermolysis Bullosa, Herlitz type.

Quick Summary:

• Epidermolysis bullosa (EB) constitute a group of genetic disorders in which there is blister formation and mechanical fragility of the skin and/or mucosal membranes after minimal trauma. A minor injury, such as friction or rubbing, can separate the skin layers easily, resulting in painful skin tearing or blistering
• Junctional epidermolysis bullosa (JEB) is the most severe form of EB. In JEB, the separation takes place at the junction of the epidermis and skin. The condition is rare and typically inherited in an autosomal dominant manner. There are two subtypes of JEB, namely:
  • Junctional Epidermolysis Bullosa, Herlitz type
  • Junctional Epidermolysis Bullosa, Non-Herlitz type
• Junctional Epidermolysis Bullosa, Herlitz type is a very severe and often fatal form of JEB. It is inherited in an autosomal recessive manner. The condition can lead to severe symptoms involving the skin and internal organs. Children with JEB, Herlitz type are not known to survive beyond infancy or early childhood (typically 24 months), despite intensive treatments

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