Alopecia-epilepsy-pyorrhea-intellectual disability syndrome

Orpha code: 1008OMIM code: 104130

Definicja

A rare genetic syndromic intellectual disability that is characterized by congenital permanent alopecia universalis, intellectual disability, psychomotor epilepsy and periodontitis (pyorrhea). Total permanent alopecia and pyorrhea are invariably concomitant while intellectual disability and psychomotor epilepsy are observed in most patients. No other abnormality of nails or skin (apart from absence of hair) has been reported. Transmission is autosomal dominant.

Disease data
Klasyfikacja

Disease

Synonimy
Shokeir syndrome
Zespół Shokeira
Kod ORPHA
1008
Kod OMIM
104130
Kod ICD10
Q87.8
Kod ICD11
LD90.Y

No additional description.

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