Late-onset localized junctional epidermolysis bullosa-intellectual disability syndrome

Orpha code: 231556OMIM code: 226440

Definicja

A rare junctional epidermolysis bullosa subtype characterized by late-onset blistering surrounded by erythema and localized on the anterior aspect of the lower legs, associated with dystrophic toenails, tooth enamel defects and mild to severe intellectual disability. Lens subluxation and mild facial dysmorphism (with short midface, prognatism and thin upper lip vermilion) are additional reported features. There have been no further descriptions in the literature since 1992.

Disease data
Klasyfikacja

Disease

Synonimy
Late-onset localized JEB-intellectual disability syndrome
Late-onset localized JEB-intellectual disability syndrome
Kod ORPHA
231556
Kod OMIM
226440
Kod ICD10
Q81.8
Kod ICD11
-

No additional description.

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