Junctional epidermolysis bullosa definition - medical term
A blistering skin condition inherited
in an autosomal recessive manner, due to mutation of a gene that normally promotes
the formation of anchoring filaments (thread-like fibers) or hemidesmosomes (complex
structures composed of many proteins). These structures anchor the epidermis to
the underlying basement membrane. The defect leads to tissue separation and blistering
in the upper part of the basement membrane.
J unctional epidermolysis bullosa (JEB) is usually severe. In the most serious
forms, large, ulcerated blisters on the face, trunk, and legs can be life-threatening
due to complicated infections and loss of body fluid that leads to severe dehydration.
Survival is also threatened by blisters that affect the esophagus, upper airway,
stomach, intestines, and the urogenital system. Other signs found in both severe
and mild forms of JEB include rough and thickened or absent fingernails and toenails;
a thin appearance to the skin (called atrophic scarring); blisters on the scalp
or loss of hair with scarring (scarring alopecia); malnutrition and anemia; growth
retardation; involvement of soft tissue inside the mouth and nose; and poorly formed
tooth enamel.
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