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

More general terms

• ichthyosis; erythrokeratodermia variabilis

Database

• OMIM: https://mirror.omim.org/entry/607602