Autosomal dominant ichthyosis
| Classification according to ICD-10 | |
|---|---|
| Q80.0 | Ichthyosis vulgaris |
| ICD-10 online (WHO version 2019) | |
The autosomal dominant ichthyosis (ADI) is the most common form of ichthyosis .
Synonyms are: Ichthyosis vulgaris ; Fish scale disease; autosomal dominant ichthyosis vulgaris (ADI)
distribution
The frequency is given as 1 in 200 to 400 children, inheritance is autosomal dominant .
root cause
The disease are mutations in FLG - gene on chromosome 1 locus q21.3 basis that the protein filaggrin coded.
Mutations in this gene can also be found in atopic eczema .
Such eczema develops in around half of those affected, in around 20% other atopy such as allergic rhinitis and bronchial asthma .
Clinical manifestations
Clinical criteria are:
- Decreased sweat production
- Sebostasis
- Very variable, well-defined, light gray, more rarely brown-black scales, symmetrical with emphasis on extremities
- Extensor sides of the legs affected, elbows, armpits, groin, hollows of the knees spared
- Increased hand line drawing "Ichthyose hand"
Differential diagnosis
A distinction must be made between X-linked recessive ichthyosis , acquired forms of ichthyosis and atopic eczema.
history
The oldest description probably comes from the year 1777 by the French doctor Anne Charles Lorry (1726–1783).
Jean-Louis Alibert published a description in 1806, followed by Robert Willan in 1808.
literature
- W. Küster: Ichthyoses: Suggestions for an improved therapy. In: Deutsches Ärzteblatt 2006; Vol. 103, No. 24, A-1684 / B-1437 / C-1389
Individual evidence
- ↑ Entry on Ichthyosis vulgaris in Flexikon , a Wiki of the DocCheck company
- ↑ a b c d e Encyclopedia Dermatology
- ↑ Ichthyosis vulgaris. In: Online Mendelian Inheritance in Man . (English)
- ^ AC Lorry: Tractatus de morbis cutaneis. P. Guillelmum Cavelier, Paris, pp. 167-184.
- ↑ L. Alibert: Descriptions des maladies de la peau, observés a l'hópital Saint-Louis et exposition des meilleurs méthodes suivies pour leur traitement. Paris.
- ^ R. Willan: Ichthyosis. In: On cutaneous disease. Barnard, London, 1808, vol. 1, chap. 4th
