Exfoliative ichthyosis is an inherited, non-syndromic, congenital ichthyosis characterized by the infancy-onset of palmoplantar peeling of the skin (aggravated by exposure to water and by occlusion) associated with dry, scaly skin over most of the body. Pruritus and hypohidrosis may also be associated. Well-demarcated areas of denuded skin appear in moist and traumatized regions and skin biopsies reveal reduced cell-cell adhesion in the basal and suprabasal layers, prominent intercellular edema, numerous aggregates of keratin filaments in basal keratinocytes, attenuated cornified cell envelopes, and epidermal barrier impairment.
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superficial epidermolytic ichthyosis
MONDO:0007813
autosomal recessive congenital ichthyosis 1
MONDO:0009441
autosomal recessive congenital ichthyosis 4A
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autosomal recessive congenital ichthyosis 11
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autosomal recessive congenital ichthyosis 5
MONDO:0011485
peeling skin syndrome 4
MONDO:0011937