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ichthyosis annular epidermolytic (cyclic ichthyosis with epidermolytic hyperkeratosis)
Pathology:
- skin disorder
Genetics:
- associated with defects in KRT1, KRT10
Clinical manifestations:
- resembles bullous ichthyosiform erythroderma
- affected individuals present with bullous ichthyosis in early childhood & hyperkeratotic lichenified plaques in the flexural areas & extensor surfaces at later ages
- episodes of flares of annular polycyclic plaques with scale, which coalesce to involve most of the body surface; flares can persist for several weeks or even months
Differential diagnosis:
- resembles bullous ichthyosiform erythroderma; ichthyosis annular epidermolytic is distinguished by episodes of flares of annular polycyclic plaques with scale
General
ichthyosis; erythrokeratodermia variabilis
Database Correlations
OMIM 607602
References
OMIM :accession 607602