Ehler's Danlos syndrome

EDS is characterized by hyperelasticity of the skin and hypermobile joints. The incidence may be about 1 in 5000 births, although a higher value has been reported for blacks. Types I, II, and III account for most diagnoses.

Biochemical assays and gene analyses for known molecular defects in EDS are difficult and time-consuming, but specific diagnostic tests should be available in the future for families in which the genes at fault have been defined.

Skin and Ehler's Danlos syndrome

The changes vary from thin and velvety skin to skin that is either dramatically hyperextensible ("rubber man" syndrome) or easily torn or scarred. Type I patients develop characteristic "cigarette-paper" scars. In type IV extensive scars and hyperpigmentation develop over bony prominences, and the skin may be so thin that subcutaneous blood vessels are visible. In type VIII the skin is more fragile than hyperextensible, and it heals with atrophic, pigmented scars. Easy bruisability occurs in several types of EDS.

Types of Ehler's Danlos syndrome

1. Type I is the classic, severe form of the disease, with both severe joint hypermobility and skin that is velvety in texture, hyperextensible, and easily scarred.
2. Type II is similar to type I but milder.
3. In type III joint hypermobility is more prominent than skin changes.
4. In type IV the skin changes are more prominent than joint changes.
5. Type IV patients are predisposed to sudden death from rupture of large blood vessels or other hollow organs. Type V is similar to type II but is inherited as an X-linked trait

Treatment of Ehler's Danlos syndrome eds

There is no specific therapy. Surgical repair and tightening of joint ligaments require careful evaluation of individual patients, as the ligaments frequently do not hold sutures. Patients with easy bruisability should be evaluated for other bleeding disorders.

Amyloidosis
Connective tissue naevi
Dermatofibroma
Dermatofibrosarcoma protuberans
Ehler's Danlos syndrome
Elastosis perforans serpiginosa
Keloids & hypertrophic scars
Lipodystrophy
Lipoma
Mastocytoma
Neurofibroma
Pseudoxanthoma elasticum
Reactive perforating collagenosis
Striae (stretch marks)
Eosinophilic fasciitis
Erythema multiforme
Histiocytoses
Jessner's lymphocytic infiltrate
Langerhans cell histiocytosis
Lymphocytoma cutis
Lymphomatoid papulosis
Mastocytosis
Morphoea
Non-Langerhans cell histiocytosis
Panniculitis
Reticulohistiocytosis
Sweet's disease
Systemic sclerosis
Atypical mycobacterial infection
Granuloma faciale
Granuloma annulare
Cutaneous tuberculosis
Leprosy
Pyogenic granuloma
Sarcoidosis