Ichthyose

Ichthyosis is a heterogeneous set of dermatologic conditions marked out by their phenotype of diffuse dryness of the skin. Ichthyosis is usually inherited, but rarely maybe acquired.

Epidemiology

Inherited ichthyosis (II) accounts for the majority of cases.  Ichthyosis vulgaris, the commonest form has an estimated prevalence in UK schoolchildren of 1 in 250 .

Acquired Ichthyosis (AI) is rare and occasionally a paraneoplastic manifestation.

Associated diseases
Malignancy
  • non-Hodgkin lymphoma
  • Kaposi sarcoma
  • multiple myeloma
  • leiomyosarcoma
  • breast
  • ovarian
  • cervical
Non-malignancy
  • autoimmune
  • systemic inflammatory disease
  • endocrine/metabolic disorders
  • HIV infection
  • medication-related

Clinical presentation

Generalized scaling and dryness of the skin with flaking and itching. On visual inspection is a classic phenotype.

Pathology

Inherited ichthyosis is divided into syndromic and non-syndromic forms. Mutations have now been found in more than 25 different genes .

Radiographic features

content pending

Treatment and prognosis

Generally symptomatic.

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