ichthyosis
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.
Siehe auch:
- Lamelläre Ichthyose
- Epidermolytische Ichthyose
- Exfoliative Ichthyose
- Ichthyosis hystrix
- X-chromosomal-rezessive Ichthyose
- Autosomal-dominante Ichthyose
- Ichthyosis hystrix Curth-Macklin
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