Table 1 Clinical classification of congenital ichtyosis (modified by Oji V. et al., 2010) [4]

Common ichthyoses

X-linked ichthyosis syndromes

 Ichthyosis vulgaris

Recessive X-linked ichthyosis

 Recessive X-linked ichthyosis non-syndromic presentation

Ichthyosis follicularis-atrichia-photophobia syndrome

Autosomal recessive congenital ichthyosis

Conradi-Hünermann-Happle syndrome

 Major types

Autosomal ichthyosis syndromes (with) Prominent hair abnormalities

  Harlequin ichthyosis

Netherton syndrome

  Lamellar ichthyosis

Ichthyosis hypotrichosis syndrome

  Congenital ichthyosiform erythroderma

Ichthyosis-hypotrichosis-sclerosing cholangitis syndrome

 Minor variants

Trichothiodystrophy

  Self-healing collodion baby

Prominent neurologic signs

  Acral self-healing collodion baby

Sjögren-Larsson syndrome

  Bathing suit ichthyosis

Refsum syndrome

Keratinopathic ichthyosis

Mental retardation-enteropathy-deafness-neuropathy-ichthyosis-keratodermia syndrome

 Major types

Severe evolution

  Epidermolytic ichthyosis

Gaucher syndrome type 2

  Superficial epidermolytic ichthyosis

Multiple sulfatase deficiency

 Minor variants

Cerebral dysgenesis-neuropathy-ichthyosis-palmoplantar keratoderma syndrome

  Annular epidermolytic ichthyosis

Arthrogryposis-renal dysfunction-cholestasis syndrome

  Ichthyosis Curth-Macklin

Other associated signs

  Autosomal recessive epidermolytic ichthyosis

Keratitis-ichthyosis-deafness syndrome

  Epidermolytic nevi

Neutral lipid storage disease with ichthyosis

Other forms

Ichthyosis prematurity syndrome

 Loricrin keratoderma

 Erythrokeratodermia variabilis

 Peeling skin disease

 Congenital reticular ichthyosiform erythroderma

 Keratosis linearis-ichthyosis congenita-keratoderma