Behcet's disease is a chronic, relapsing vasculitis affecting multiple organ systems. It is characterized by recurrent oral and genital ulcers, eye lesions, skin lesions, and arthritis. The cause is unknown but is believed to involve infectious agents triggering an autoimmune response in genetically susceptible individuals. Treatment involves managing symptoms with medications tailored to specific organ manifestations, with the goal of reducing exacerbations and preventing long term complications like blindness or vascular damage.

1. Presentor:Dr Chanabasayya Viraktamath
Moderator:Dr Sukumar D

2. Historical aspects:
In 1937, Prof. Hulusi
Behçet, a Turkish
dermatologist (1889-
1948), described a
syndrome characterized by
recurrent oral ulcers,
genital ulcers, &
hypopyon uveitis of
unknown cause.

3. Behcet’s disease is a chronic, relapsing,
occlusive vasculitis affecting multiple organ
systems.

4.  World wide occurrence with varying prevalence.
 Endemic along the Silk Road(Eastern and Central
Asian and the Eastern Mediterranean countries).

5.  Prevalence rate:(per 100000 population)
 Turkey:110-420.
Israel:50-185.
Japan:13-20.
 Male preponderance.
 Age group-20-35 years.

6.  Etiology unknown.
 Caused by certain environmental factors (infectious
agents) in genetically susceptible host.
 In endemic areas ,strongly associated with HLA-B51
gene.
 Also linkage disequilibrium of HLA-B51 with
MICA*009 and TNF-1031C genes.

7.  Other genes causing genetic susceptibility are those
coding for ERAP-1,
IL-23R,
IL-12B2R,
Coagulation factor-V,
Endothelial nitric oxide synthase,
Intercellular adhesion molecule-1.

8.  Behcet’s disease is non contagious.
 VIRAL:
 HSV-1,
 Hepatitis virus,
 Parvo virus- B19,
 BACTERIAL:
Streptococcus.sanguinis,
Mycoplasma fermantans,
H.Pylori,
Staph.aureus,
Saccharomyces cervisiae

9. Infective agents trigger auto immune
response against self antigens.
Antigens against which Auto Ab found are,
Endothelial antigen(alpha enolase),
Retinal S antigen,
Heat Shock proteins(60 & 70 Kda),
Tropomyosin,
Costimulatory molecules,
Oxidized low density lipoprotein

10. Behcets disease 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.

11.  Recurrent oral &genital
ulcers,ocular lesions,skin
manifestations & arthritis
/arthropathy most frequent
clinical manifestations.
 Vascular,GIT,CNS,psychia
tric,pulmonary,renal and
cardiac
manifestations,epididymiti
s can also occurs.

12. SYMPTOM - 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
BCD scoring: Score>4 indicates Behcet disease

13. • 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

14. 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 –
(1) 2 major features or,
(2) 1 major and 2 minor features

15. Recurrent oral ulcers-
 Presenting sign in >90% of
cases.
 Recur(atleast 3 episodes in a
year)
 Grossly & histologically
similar to common oral
ulcers, but are more
extensive and multiple ulcers.
 Lesions are
multiple,painful,1-3 cm in
diameter & sharply margined
with fibrin coated base and
surrounding erythema.
 Heals without scarring in 4-
30 days.

16. • Genital ulcers (90%,
M>F) resemble their oral
counterparts but cause
greater scarring.
• In males, usually occur on
scrotum, penis, and groin.
• In females, occur on
vulva, vagina, groin, and
cervix
• Ulcers may found in
urethral orifice and
perianal area.
• Epididymitis may arise.

17.  47-65% of cases
 Major cause of morbidity.
 Ocular lesions are,
Anterior uveitis
Posterior uveitis(Retinal vasculitis)-lead to blindness.
Hypopyon.
Secondary complications(Cataract,Glaucoma,Tractional
retinal detachment,chronic cystoid macular edema,vision
loss & Neovascular lesions)
 Blindness occur within 4-5 years from onset of ocular
symptoms.

18. Ocular involvement showing
posterior uveitis.
HYPOPYON – pus in the anterior
chamber associated with uveitis

19. These include,
Pseudofolliculitis,
Erythema nodosum like lesions,
Acneiform lesions,
Papulo pustular type hypersensitivity.

20. Papulopustular eruptions
(55-83%, M>F)
Erythema nodosum–like
lesions (44-62%, F>M)

21.  Nonerosive.
 Asymmetric
 Nondeforming
 Sterile
 Seronegative
 Oligoarthritis
 Knee,Ankle & Elbow joints commonly affected.

22. • Neutrophilic systemic vasculitis
• Both Arteries & Veins of all calibers affected.
Manifestations are,
Superficial thrombophlebitis,
Deep vein thrombosis,
Arterial occlusions,
Arterial aneurysms.

23. Gastrointestinal involvement (10%)
• Ileocecal ulcers
• Anorexia, vomiting, dyspepsia, diarrhea, abdominal
distention, and abdominal pain
• Cardiac manifestations (5-17%)
• Coronary vasculitis and thrombosis, pericarditis,
myocarditis, endocarditis.

24. • Lung involvement (up to 18%)
• Pulmonary vasculitis, hypertension, pleural effusions
and Aneurysms
• Neurological manifestations(10 %):
• Meningoencephalitis,
• Cerebral venous sinus thrombosis,
• Benign intracranial hypertension,
• Cranial nerve palsies,
• Brainstem lesions

25.  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

26.  Characteristic features are
vasculitis & thrombosis.
 Neutrophilic vascular
reaction with endothelial
swelling (predominant
finding)
 Extravasation of
erythrocytes,
 Leukocytoclasia/fully
developed leukocytoclastic
vasculitis
 Fibrinoid necrosis of blood
vessel walls
 Lymphocytic perivasculitis.

28.  PATHERGY TEST:
 Site: Commonly volar
aspect of forearm.
 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.

29.  Pathergy test is not pathognomic.
 It can also occur in patients with,
 Pyoderma gangrenosum
 Rheumatoid arthritis
 Crohn disease
 Genital herpes infection

30. • 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

31. • Treatment of BD symptomatic and empiric.
• Choice of treatment depends on site and
severity of clinical manifestations

32. 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

33. 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)

34. Pulmonary or peripheral arterial aneurysms
Cyclophosphamide
Anti– TNF
Neurological involvement:
Azathioprine(2.5 mg/kg/day)

35. • 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.

36.  IADVL text book of dermatology,4 th edition
 ROOK’s text book
 Fitzpatrick text book
 Internet sources

37.  THANK YOU