BEHCET'S DISEASE

History:

In 1937 Hulusi Behcet, a

Turkish dermatologist described
a syndrome characterized by
recurrent oral ulcer, genital
ulcer and hypopyon uveitis of
unknown case.
BEHCET'S DISEASE
 It is a chronic relapsing ,occlusive vasculitis affecting
multiple organ systems
EPIDEMIOLOGY:
 Endemic along the Silk Road(Eastern and Central Asian
and the Eastern Mediterranean countries).
PATHOGENESIS
 Etiology unknown.
 Caused by certain environmental factors
(infectious agents) in genetically susceptible host.
 In endemic areas ,strongly associated with HLA-
B51gene.
 Antibodies against alpha enolase of endothelial
cells.
 IL-10, IL- 23R, IL-12B2R association is also seen.
 The environmental trigger hypothesis has also
been proposed in BD patients with genetic
susceptibility.
 Behcet's disease is both autoinflammatory
and autoimmune disorder.
 Autoinflammatory refers to inherited
disorders with episodes of recurrent
inflammatory reactions of the innate
immune system without remarkable
provocation.
 Autoimmunity refers to significant levels of
high titer autoantibodies or antigen specific
T cells.
 CLINICAL FEATURES
 Recurrent oral
ophthous ulcer.
 genital ulcers
 ocular lesions.
 skin manifestations &
arthritis/arthropathy
most frequent clinical
manifestations.
 Vascular, GIT, CNS,renal,
psychiatric, pulmonary,
and cardiac manifestation
s can also occurs.
Cutaneous manifestations includes,
 Pseudofolliculitis,
 Erythema nodosum like lesions,
 Acneiform lesions,
 Papulo pustular type
hypersensitivity.
Erythema nodosum–like Papulopustular eruptions
lesions (44-62%, F>M) (55-83%, M>F)
 ARTHRITIS
 Nonerosive.
 Asymmetric
 Nondeforming
 Sterile
 Seronegative
 Oligo arthritis
 Knee, Ankle & Elbow joints commonly
affected.
 VASCULAR INVOLMENT

• Neutrophilic systemic vasculitis

• Both Arteries & Veins are affected.
Manifestations are,
 Superficial thrombophlebitis,
 Deep vein thrombosis,
 Arterial occlusions,
 Arterial aneurysms.
GASTRO INTESTINAL INVOLVEMENT

• Ileocecal ulcers
• Anorexia, vomiting, dyspepsia, diarrhea,
abdominal distention, and abdominal pain
• Cardiac manifestations (5-17%)
• Coronary vasculitis and thrombosis, pericarditis,
myocarditis, endocarditis.
LUNG INVOLVEMENT

 Pulmonary vasculitis, hypertension, pleural effusions and

Aneurysms

Neurological manifestations(10 %):

 Meningoencephalitis,
 Cerebral venous sinus thrombosis,
 Benign intracranial hypertension,
 Cranial nerve palsies,
 Brainstem lesions
DIAGNOSTIC
CRITERIA
• Major features(4)
• Recurrent ulceration of oral mucous membrane
• Skin lesions - Erythema nodosum–like lesions,
superficial thrombophlebitis, papular skin hypersensitivity
• Eye lesions – Iridocyclitis or its sequele,
-Posterior uveitis or its sequele
• Genital ulcers

• Minor features(5)
• Arthritis without deformity or sclerosis
• Gastrointestinal lesions characterized by ileocecal ulcers
• Epididymitis
• Vascular lesions
• Central nervous system symptoms moderate or severe
 DEPENDING ON CRITERIA,

a) Complete –
Four major features

b) Incomplete –
(1) 3 major features or,
(2) 2 major and 2 minor features or,
(3) Typical ocular symptom and 1 major or 2 minor features

c) Possible –
(4) 2 major features or,
(5) 1 major and 2 minor features
INTERNATIONAL CRITERIA FOR
BD
SYMPTOMS POINTS
• Ocular lesions(recurrent) 2
• Oral ulcers(recurrent) 2
• Genital ulcers(recurrent) 2
• Skin lesions(recurrent) 1
• CNS symptoms 1
• Vascular manifestations 1
• Pathergy test(+ve) 1
Total -

10
 Complete blood count: Increased(neutrophilia)
 C –reactive protein- raised
 Beta 2 microglobulin-raised
 Serum myeloperoxidase-raised
 LFT-raised(if liver involved)
 Skin Biopsy
 Urine analysis- Proteinuria,hematuria(if kidney involved)
 Barium studies
 CSFanalysis
 MRI/CT Scan
 Chest X ray
 Endoscopy
 Pathergy test
 PATHERGY TEST:

 Intradermal injection of 0.1

ml isotonic salt solution
using 20 G needle without
prior disinfection of the
injection site.
 3-5 mm intradermally at an angle
of 45 degree.
 Reading – after 24-48 hours
 +ve result- Erythematous papule
or pustule (>2 mm) at prick site.
• Sweet’s syndrome
• Bullous autoimmune
diseases
• Reiter disease
• Ulcerative colitis, crohn
disease
• Erythema Multiforme
• Viral infections
• Herpes Simplex, coxsackie
• Syphilis and other STIs
• SLE
• Other connective tissue
disease
MANAGEMENT:
 Treatment of BD symptomatic and empiric.
Choice of treatment depends on the site and
severity of clinical manifestations
Mucous membrane inv:
 Topical glucocorticoids (Triamcinolone
acetonide in orabase/ prednisolone 5 mg in
20 ml water/ tetracycline 250 mg in 20 ml
water used as mouth wash TID)
 Topical anesthetics(Lidocaine 2%
/tetracaine 0.5-1% in gel form)
 Topical
immunosuppressant( Cyclosporine
solution)
 Serious cases- thalidomide
Arthritis:
 Colchicine(1-2 mg/day)
 Azathioprine(2.5 mg/kg/day)

Thrombophlebitis
 Aspirin

Uveitis:
 Systemic steroids( Methyl prednisolone 40 mg
IM once in 3 wks)
 Azathioprine(2.5 mg/kg/day)
 Cyclosporin(2-10 mg/kg/day)
 PROGNOSIS

• Behcet's disease has an undulating course of

exacerbations and remissions, and may
become less severe after approximately 20
years.
• Appears to be more severe in young, male,
and Middle Eastern or Far Eastern patients.
REFERENCES:

 https://www.frontiersin.org/articles/10.3389/fimmu.2019.00
665/full
 Harrison's internal medicine text book
 https://www.neurovascularmedicine.com/behcet.php
 https://www.hopkinsvasculitis.org/types-vasculitis/behcets-d
isease/
 YOU
N K
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