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Not always true…
Dr Aisling Loy GUM SPR
Presenting history
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• DY 39 year old female
from Israel living in
Ireland 20yrs
• Referred by GP Oct 12
with several day hx of
painful mouth and
genital ulcers
• Hx of falling downstairs
1 week prior.
• Multiple bruises from
fall
History
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• Past Medical Hx of Hidradenitis
suppurativa, recurrent oral apthous
ulcers x 8years
• Fam Hx-
Hx
Lives with partner
25 pkyr Hx
Occasional C2H5OH
No children
• Father has Familial Mediterranean
Fever and Ankylosing spondylitis
• Aunt Behcets
Examination
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Normal vitals
CVS/GIT/Resp/Neuro NAD
Mouth- multiple ulcers, white coating
GU- 2 x 2cm ulcers on left labia majora.
Indurated and tender.
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Laboratory
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WCC 29.3
Neut 25
Hb 15.2
Plat 315
CRP 248
ESR 22
U/E NAD
CXR NAD
ECG NSR
Management plan
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Ulcer swabs taken
IV Augmentin/ prophylactic clexane
Tramadol/Tylex
ANA/ANCA sent
HIV serology
HSV serology
GUM and Derm consults
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Progress
• Reviewed by GUMTemp spike 38.5 and
continued to spike for
next 2 days.
• Blood cultures taken
• STI screen
• ?Behcets
• Opthalmology review
requested
• Skin Bx taken from
Vulva
Treatment
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Commenced Dermovate Topical
Beclamethasone Oral
Valtrex ?HSV
Fluconazole
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Skin Bx result
• Skin Bx- dermal
inflammation
comprising of
lymphocytes,
histocytes and
polymorphs. There
are ectatic vascular
spaces.
Results
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Xray of SI joints NAD
ANA neg
ANCA neg
CRP continued to improve
STI screen neg
HSV culture neg
Hep B/C
EBV Neg
Progress
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Discharged home after 2 weeks
Completed 7 days of IV/PO Augmentin
CRP reduced
Poor initial response to topical dermovate
Changed to Colchicine 1mg BD for 2 weeks
Followed up in OPD
Acute inflammation of the apocrine glands
Special stains for Herpes virus, fugi,
Mycobacteria and bacteris are negative.
2 weeks later in OPD
• Sig response to colchicine, not
completely healed
• Returned to Isreal reviewed by Behcet
specialist
• Continued on 1.5mgs OD
• For Exacerbation prednisolone 20mgs
• HLAB51 neg
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Dx with Behcets
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• What is Behcets?
• Why did she develop Behcets now?
• Is there a link to her family hx and her own
medical Hx?
• What will the future hold?
Hx of Behcets
• Hulusi Behcet (18891948 )
• Turkish Physician
• In 1937, described the
classic trisymptom
complex of hypopyon,
iritis, and orogenital
aphthosis
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2500yrs ago
• Hippocrates of Kos
(460-377 BC)
• Endemic disease in
Asia Minor
characterised by
“aphthous ulcerations,
defluxions about the
genital parts,watery
ophthalmies of a
chronic character . . .
which destroyed the
sight of many
persons,large herpetic
lesions.”
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Spectrum of organ involvement in Behçet’s disease.
Verity D H et al. Br J Ophthalmol 2003;87:1175-1183
©2003 by BMJ Publishing Group Ltd.
Laboratory
• Mild anemia and leukocytosis
• The ESR, C-reactive protein may be raised.
• Serum immunoglobulin levels, especially
immunoglobulin A, may be elevated.
• Circulating immune complexes.
• Rheumatoid factor and antinuclear antibodies are
absent.
Cutaneous manifestations
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Joint manifestations
• 50% develop signs or symptoms of synovitis,
arthritis, and/or arthralgia.
• The most frequent minor feature in childhoodonset BD.
• Multiple-joint involvement is common.
a dna ™emiTkciuQ
rosserpmoced
.erutcip siht ees ot dedeen era
Vascular involvement
• This occurs in 7-29% of patients, mostly men.
• The 4 types of vascular lesions arterial occlusions,
venous occlusions, aneurysms, and varices.
• Arterial complications account for 7% of cases.
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Gastrointestinal involvement
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10% of patients with BD.
Anorexia,
vomiting,
dyspepsia,
diarrhoea,
abdominal distention,
abdominal pain
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Neurologic manifestations
• Varies from 3.2-49%. (rare)
• Neurologic involvement may present in
various combinations.
• Most serious complications, high fatality rate.
• Occur within 5 years of disease onset
• Severe headache is the most frequent.
Behçet’s disease is associated with multiple hereditory and environmental risk factors.
Verity D H et al. Br J Ophthalmol 2003;87:1175-1183
©2003 by BMJ Publishing Group Ltd.
Genetics
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• HLA-B51
• Associated with Factor V Leiden
deficiency
• Displays genetic anticipation
• More common in Isreal Arabs than
Jews.
Immunological Abnormalities
• Neutrophil infiltration, endothelial cell
swelling, fibrinoid necrosis.
• Increased levels of neutrophil priming
cytokines, TNF, IL1B, IL 8,
myeloperoxidase levels
• ?genetic v primining agent v both
?Infective aetiology
• Silk road promoted the spread of
infection eg the black death, the plague
of Athens. Behcet suspected an
infectious aetiology
• Monocytes show hypersensitivity to
Streptococccal sanguis antigen
• Higher levels of HSV 1 antibodies
Behçet’s disease.
Verity D H et al. Br J Ophthalmol 2003;87:1175-1183
©2003 by BMJ Publishing Group Ltd.
Link to Familial Mediterranean
Fever
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• Gene ass’d with FMF (MEFV)
• 4 mutations ass’d with Behcets
• Gene codes for pyrin thought to regulate
neutrophils
• Similar ethnic distribution and effect on
neutrophils
• Thought these two are linked to same gene.
• Thought to be a link with hidradenitis
suppurativa
Conventional Treatment
SYSTEMIC RX
Azathiopine
Interferon alpha
Cyclophosphamide
Cyclosporin
Infliximab
Etanercept
Adalimumab
Rituximab
Anticoagulants
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Novel Treatments
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Probiotic yoghurt
Topical Tacrolimus
Sublingual interferon alpha
Zinc sulphate mouth wash
Nigella sativa oil
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Why now?
• Genetically primed plus a stress on the body starting
a cytokine/neutrophilic/lymphocytic/ hyper response
leading to a pathological systemic vasculitic
phenonomen
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Summary
• Multisystem
• Complex aeitology, both genetic and
environmental
• Varying prognosis
• No specific diagnostic test
• Treated with different methods of
immune modulation.