Buithieu

Figure 1: Luxation of the temporomandibular joint in a 12-year-old patient.

Figure 2: Bilateral bleeding in the cheeks following episodes of luxation.

Figure 3: Unusual body posture demonstrating joint hypermobility.

Figure 4: Hyperextension and hyperabduction of the fingers.

Figure 5: A panoramic radiograph demonstrating the absence of 2 maxillary premolars.

Table 1 Classification of Ehlers-Danlos syndrome

Type	Transmission	Clinical Features	Biochemical Defects	% of Cases
I – Severe or classic	AD^a	Skin hyperelasticity and fragility Dystrophic scars Joint hypermobility Abnormal bleeding tendency	Unknown	80%
II – Moderate	AD	Symptoms less pronounced than in type I	Unknown	80%
III – Familial hypermobility	AD	Joint hypermobility Multiple dislocations Skin hyperelasticity Absence of dystrophic scars Normal coagulation	Unknown	10%
IV – Vascular (Sack-Barabas) A, B, C, D	AD (A, B, C) AR^b (D)	Vascular fragility (arterial rupture) Moderate hypermobility of the finger joints Perforation of certain hollow organs (uterus, intestine)	Deficit of collagen type III	4%
V – Chromosome X linked	XL^c	Similar to types II and III	Unknown	» 6%^d
VI – Ocular A, B	AR (A, B)	Hyperelasticity of the skin Joint hypermobility Detached retina Severe scoliosis	Lysyl hydroxylase deficiency
VII – Arthrochalasis multiplex congenita A, B, C	AD (A, B) AR (C)	Congenital luxation of the hips Multiple joint dislocations Moderate hyperelasticity of the skin Small stature	A - Faulty pro-alpha 1 structure B - Faulty pro-alpha 2 structure C - Procollagen n-proteinase deficiency
VIII – Periodontal	AD	Generalized early-onset periodontitis Moderate joint hypermobility Variable skin hyperelasticity Shin ecchymoses	Unknown

^aAD Autsomal dominant

^bAR Autosomal recessive

^cXL Chromosome X linked

^dApproximate % including all other types (V - XI, Hernandez, Friedman-Harrod, Beasley-Cohen, Viljoen)

Table 2 Clinical manifestations of Ehler-Danlos syndrome

Examination	Clinical Signs	Complications
Extraoral	Scarring of the forehead and chin Hypertelorism and epicanthus Narrow curved nose Thin hair Deformed ears	Frequent luxations and subluxations of the TMJ Hyperelasticity of the skin
Intraoral (soft tissues)	Supple tongue Gingival hyperplasia and fibrous nodules²¹	Buccal mucosa: Fragility Reduced healing capacity Bruising tendency Periodontal tissues: Bleeding after brushing Vulnerable to gingivitis and periodontal disease Excessive post-extraction bleeding
Intraoral (hard tissues)	Vaulted palate Deep fissures and long cusps Microdontia Supernumeraries²² Pulp calcification Abnormal roots	Teeth: Fragility Tendency to fracture

Clinical Features