Late-onset junctional epidermolysis bullosa

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Synonyms: Epidermolysis bullosa progressiva | JEB-lo | Late-onset JEB

A form of junctional epidermolysis bullosa characterized by onset in childhood or young adulthood of blistering that first occurs around nails accompanied by nail dystrophy and shedding and then affects the hands and feet and to a lesser extent the elbows and knees. Lesions heal with atrophic scarring. Other manifestations include disappearance of dermatoglyphs and palmoplantar hyperhidrosis. Extracutaneous involvement is restricted to soft tissue abnormalities of the oral cavity and enamel defects with development of caries.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version July 2024

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Late-onset junctional epidermolysis bullosa?

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