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ORPHA:477Disease
Also called Ichthyosis hystrix Rheydt type · KID/HID syndrome · Keratitis-ichthyosis-deafness/Hystrix-like ichthyosis-deafness syndrome · Keratitis-ichthyosis-hearing loss/Hystrix-like ichthyosis-hearing loss syndrome · Senter syndrome
What it is
A rare congenital ectodermal disorder characterized by vascularizing keratitis, hyperkeratotic skin lesions and hearing loss.
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- Neonatal
- Inheritance
- Autosomal dominant, Autosomal recessive, Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
24- Abnormality of the dentition
- Congenital ichthyosiform erythroderma
- Conjunctivitis
- Corneal erosion
- Epidermal acanthosis
- Follicular hyperkeratosis
- Keratitis
- Keratoconjunctivitis sicca
- Limbal stem cell deficiency
- Nail dystrophy
- Palmoplantar keratoderma
- Photophobia
- Prelingual sensorineural hearing impairment
- Progeroid facial appearance
- Punctate keratitis
- Recurrent skin infections
- Scaling skin
- Scarring alopecia of scalp
- Severe sensorineural hearing impairment
- Skin plaque
- Sparse eyebrow
- Sparse eyelashes
- Sparse hair
- Visual loss
Sometimes5–29%
14- Angular cheilitis
- Aplastic/hypoplastic lacrimal glands
- Arthritis
- Failure to thrive
- Folliculitis
- Hypohidrosis
- Neurodevelopmental delay
- Onychogryphosis
and 6 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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