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Пародонтальний синдром Елерса-Данлоса

ORPHA:75392· ICD-10 Q79.6· Periodontal Ehlers-Danlos syndrome

Визначення(English summary)

A rare type of Ehlers-Danlos syndrome characterized by childhood or adolescence onset of severe, intractable periodontitis, lack of attached gingiva, and presence of pretibial plaques. Additional manifestations are easy bruising, hypermobility mainly of the distal joints, skin hyperextensibility and fragility, abnormal scarring, recurrent infections, hernias, marfanoid facial features, acrogeria, and prominent vasculature.

Поширеність
Unknown
Успадкування
Autosomal dominant
Вік початку
Childhood