Chapter: Medicine and surgery: Musculoskeletal system

Behc¸et’s syndrome

A chronic, relapsing multisystem vasculitis characterised by oral ulceration. - Definition, Incidence, Aetiology, Pathophysiology, Clinical features, Complications, Investigations, Management, Prognosis.

Behc¸et’s syndrome

 

Definition

 

A chronic, relapsing multisystem vasculitis characterised by oral ulceration.

 

Incidence

 

Rare.

 

Age

 

Commonest in 20–40 years.

 

Sex

 

> F

 

Geography

Much more common in Turkey, Iran, China, Korea and Japan.


Aetiology/pathophysiology

 

It is thought to be the result of an environmentally triggered autoimmune reaction in a genetically susceptible individual.

 

Clinical features

 

Patients have recurrent oral aphthous or herpetiform ulcers. Other manifestations include genital ulceration, ocular disease (uveitis), skin lesions (erythema nodosum), arthritis, gastrointestinal upset, renal, lung and neuro-logical involvement. Patients demonstrate pathergy (a papule or pustule forms at sites of skin puncture) – this is a highly specific finding.

 

Management

 

Corticosteroids and immunosuppressive agents are used for severe disease. Colchicine may be of benefit for erythema nodosum and arthralgia.

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Medicine and surgery: Musculoskeletal system : Behc¸et’s syndrome |

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Medicine and surgery: Musculoskeletal system


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