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Kindler syndrome (hereditary acrokeratotic poikiloderma)
Epidemiology:
1) rare
2) infants, children, may persist into adulthood
Genetics:
- mutation in the KIND1 gene
Clinical manifestations:
expression variable, 4 patterns suggested:
1) vesicopustule formation remains confined to the hands & feet
- begins 1-3 months of age, resolves in late childhood;
2) widespread eczematoid dermatitis resembling atopic eczema
- starts 3-6 months of age, completely resolves by age 5
3) gradual appearance of diffuse poikiloderma with striate & reticulate atrophy
- spares ace, scalp, & ears
- persists into adulthood
4) development of keratotic papules on the hands, feet, elbows, & knees
- lesions first appear at varying times before 5 years of age & persist indefinitely.
Related
KIND1 gene
General
genetic syndrome (multisystem disorder)
poikiloderma
Database Correlations
OMIM 173650
References
OMIM :accession 173650