Bechet’s Syndrome

Hypopyon REF: Khurana 4th ed p. 156

Uveitis in bechet’s disease

• Bilateral
• Recurrent iridocyclitis
• Hypopyon
• Viteritis
• Periphlebitis
• Retinitis

The eye disease is present in 50% of patients with Bechet’s syndrome. It is usually present at the onset but may also develop within the first few years. 

Iritis, posterior uveitis, retinal vessel occlusions and optic neuritis can be seen in some patients with the syndrome.

Note: Scarring and bilateral panuveitis is the most dreaded complication of Bechet’s, since it occasionally progresses rapidly to blindness.

Ref: Moutsopoulos H.M. (2012). Chapter 327. Behçet’s Syndrome. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds), Harrison’s Principles of Internal Medicine, 18e.

Ans. is ‘c’ i.e., HLA-B51

B i.e. Bechet’s syndrome

Recurrent oral apthous ulcers (oral ulcers with yellow floor surrounded by erythematous halo) with erythema nodosum like nodules (multiple, tender nodules on shin) suggest the diagnois of Behcet’s syndrome.

A i.e. Becht’s syndrome, C. i.e. Reiter’s syndrome

Reiter’s syndrome (reactive arthritis) is an acute nonpurulent, asymmetrical, additive (poly) arthritis triggered by an infection elsewhere (like STD, enteric infection because of travel to endemic area), HIV & HLA B 27Q. It presents with classical triad of “CUP” i.e. conjunctivitis, urethritis and polyarthritis Q. Dactylitis (sausage digit), enthesitis, asymmetrical spondylitis & sacroilitis, onycholysis & hyperkeratosis of nails, circinate balanitis of glans, keratodernia blenorrhagica on palms & soles and superficial asymptomatic aoral ulcers are other features of reactive arthritis.

D i.e. HIV associated neuropathy

Peripheral neuropathy is a dose limiting toxicity of thalidomide & not a therapeutic indicationQ. It causes painful sensory ganglionopathy & axonal neuropathy.

Indications of Thalidomide

– Erythema nodosum leprosum (ENL)Q: Only FDA approved use for treatment of cutaneous manifestations . of moderate to severe ENL and for maintenance therapy for prevention and suppression of cutaneous manifestations of ENL recurrence.

– Severe, recurrent apthous stomatitisQ, especially in AIDS patients is common off label use. – Behects syndromeQ

– Chronic graft versus host disease.

* HIV associated colitis and wasting, multiple myeloma, myelodysplasia, chronic idiopathic myelofibrosis, glucocorticosteroid refractory fistulosis-Chron’s disease, sarcoidosis, prurigo, stomatitis are other rare uses.

Ans. is ‘b’ i.e., Bechet’s syndrome

Criteria for diagnosis of Bechet’s disease

• Recurrent oral ulcer for at least 3 times in 12 months.
• Plus any two of the followings :‑

1. Recurrent genital ulcer.
2. Eye inflammation with loss of vision.
3. Characterstic skin lesion (erythema nodosum).
4. Positive pathergy test (skin prick test).

Ans. is ‘c’ i.e., HLA-B51