Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition 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. Disease data Classification Disease Synonyms EDS VIII Zespół Ehlersa i Danlosa typ okołozębowy Ehlers-Danlos syndrome type 8 Ehlers-Danlos syndrome, periodontitis type Periodontal EDS pEDS ORPHA code 75392 OMIM code 130080 ICD10 code Q79.6 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl