Conditions / Skin

autosomal recessive congenital ichthyosis 4A

info · Skin · ICD-10: Q80.2

An autosomal recessive congenital ichthyosis characterized by generalized ichthyosis and ectropion that has_material_basis_in homozygous or compound heterozygous mutation in the ABCA12 gene on chromosome 2q35.

Signs and symptoms

  • Congenital nonbullous ichthyosiform erythroderma
  • Ectropion
  • Palmoplantar keratoderma
  • Clubbing
  • Leukonychia
  • Spastic paraplegia
  • Hepatosplenomegaly

Also known as: ARCI4A; ICR2B; ichthyosis congenita IIB; lamellar ichthyosis 2