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Behcet’s Disease

Title               

Ñ    Behcet’s Disease

Definition

Ñ    Inflammatory disease of unknown cause

Characterised by:

Ñ    Recurrent oral ulceration

With:

Ñ    Genital ulceration

Ñ    Skin lesions

Ñ    Eye lesions

Ñ    Positive Pathergy test                        

Pathogenesis

Causes                 

Ñ    Cause unknown

Ñ    Epidemiology:

Common in Japan and countries bordering the Mediterranean Sea

Ñ    Immunogenetics:

·          HLA-B51:

Significant association

·          HLA-B51, B 12, DR7, DR2:

May be associated with tissue localisation

Pathological Process

Ñ    Lymphocytic vasculitis

Ñ    Skin lesions secondary to small vessel vasculitis

Clinical Features

Diagnostic Criteria

The international study group criteria for the diagnosis of Behcet’s disease  are:

Ñ    Recurrent oral ulcers:

Minor aphthous

Major aphthous

Herpetiform ulcers

Recurred at least three times a year

Plus any two of the following:

Ñ    Recurrent genital ulcers:

Ulcers or scarring

Ñ    Eye lesions:

Anterior uveitis

Posterior uveitis

Cells in the vitreous on slit lamp examination

Retinal vasculitis

Ñ    Skin lesions:

Erythema nodosum

Pseudofolliculitis

Papulopustular lesions

Acneiform lesions in post adolescents not on corticosteroids

Ñ    Positive pathergy test:

Skin injury by needle prick leads to papule or pustule formation in 24-48 hours

History

Age

Ñ    Common in 3^rd decade

Sex

Ñ    Males > Females

E&M

Ñ    Malaise

Ñ    Fever

Ñ    Loss of weight:

Occasionally with acute exacerbation

GIT

Ñ    Anorexia

Ñ    Nausea

Ñ    Distension

Ñ    Diarrhoea

Examination

IS

Ñ    Diffuse pustular lesions

Ñ    Erythema nodosum

Ñ    Erythema multiforme

GIT

Ñ    Oral ulcers:

Recurrent major or minor aphthous ulcers, herpetiform ulcers

Ñ    Perianal ulcers

RAG

Ñ    Genital ulcers

CNS

Ñ    Eyes:

Uveitis, iridocyclitis, retinal vascular lesions, optic atrophy

Ñ    Brainstem syndromes

Ñ    Cerebral lesions

Ñ    Spinal cord lesions

LMS

Ñ    Arthritis or arthralgia

CVS

Ñ    Thrombophlebitis of leg veins

Ñ    Aneurysms

Investigations

Fluids

Blood

Immunogenetics

Ñ    HLA B 12, DR 2:

Elevated in mucocutaneous type

Ñ    HLA B 12 DR 2:

Elevated in arthritic type

Ñ    HLA DR 7, DRw52:

Elevated in neurological type

Ñ    HLA B51, DR 7:

Elevated in ocular type

Physiological tests

Pathergy Test

Ñ    Sterile subcutaneous injection (without any material):

Produces a papule or pustule within 24-48 hours

Management

Control

Drugs

Ñ    Steroids:

Local or systemic

Ñ    Immunosuppression

Ñ    Colchicine:

Erythema nodosum

Joint involvement

Ñ    Thalidomide

Ñ    Tetracycline:

Locally

 

 

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