Alopecia-epilepsy-pyorrhea-intellectual disability syndrome

Orpha code: 1008OMIM code: 104130

Definition

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
Classification

Disease

Synonyms
Shokeir syndrome
Zespół Shokeira
ORPHA code
1008
OMIM code
104130
ICD10 code
Q87.8
ICD11 code
LD90.Y

No additional description.

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