Table 1.

clEDS2 reported clinical features in this report and their overall frequencies in published cases.

Clinical Features	Reported in	Features of this Case
Mucocutaneous Features
Cutaneous hyperextensibility	10/10	+
Easy bruising	10/10	+
Redundant skin	8/10	+
Delayed wound healing	8/10	−
Atrophic scarring	7/10	+
Translucent skin	6/10	+
Progeroid aspect hands/feet	6/10	+
Helicitable piezogenic pedal papules	4/10	+
Paradontal/tooth anomalies a	4/10	+
Hair anomalies b	4/10	−
Soft doughy texture	2/10	−
Musculoskeletal features
Hypermobility c	9/10	+
Foot deformities d	10/10	+
Dislocation/subluxations e	9/10	+
Scoliosis/kyphosis	7/10	+
Other anomalies of the spine f	4/10	−
Osteopenia/osteoporosis	5/10	−
Early onset arthrosis g	4/10	−
Pectus excavatum	3/10	−
Inguinal/umbilical hernias	4/10	+
Muscular involvement h	3/10	+
Perinatal hypotonia	3/10	+
Cardiovascular Features
Cardiac valves anomalies i	5/10	+
Hematomas	3/10	+
Varicose veins	3/10	+
Postural orthostatic tachycardia syndrome	2/10	−
Other Features
Preterm/premature birth	5/10	+
Bilateral cryptorchidism	3/6	+
Ocular anomalies j	5/10	−
Mild facial dysmorphism k	5/10	+
Delays motor development	5/10	+

^a Periodontal disease (2), bad tooth qualities (enamel defects), and multiple caries (3); ^b alopecia (3), thinning of the hair (2); ^c generalized joint hypermobility (9), localized hypermobility (1) reported only in patient D-II in whom BS was assessed at age 38 (see reference [5]); ^d hallux valgus (6), hammer toes (5), pes planus (8), hindfoot deformities (2), aplasia/hypoplasia of the distal phalanges of the feet digits (1); ^e shoulder (7), knees (4), elbow (3), wrist (2), hip (3), ankle (3), finger (2); ^f vertebral anomalies (3), disc bulging (2); ^g spine (2), knee (1), temporomandibular (1); ^h muscle weakness (2), right pectoralis hypoplasia (1); ⁱ 3 mitral valve prolapse, 2 mild polivalvular insufficiency; ^j myopia (4), astigmatism (3), keratoconus (1); ^k micrognathia (4), webbed neck (2), low set ears (2).