OD 34 - Bechet's disease

Question 1

What is cyclic neutropenia?

Answer 1

Periodic decrease in the circulating neutrophils
Sporadically or autosomal dominant
Developing recurrent infections
Occurs every 21 days and lasts 3-5 days

Question 2

What is PFAPA syndrome?

Answer 2

Periodic fever adenitis pharyngitis aphthous ulcer syndrome

Unknown aetiology

Question 3

What is Magic syndrome?

Answer 3

Overlap of Behcet’s and relapsing polychondritis (RP) manifestations
Sore ears/ nose/ eyes/ arthritis/ resp tract infection

Question 4

What is Behcet’s disease?

Answer 4

Multisystem inflammatory disorder
Recurrent oral ulcers, genital ulcers & ocular inflammation
Classified as vasculitis

Question 5

What is the diagnostic criteria of Behcet’s disease?

Answer 5

Recurrent oral ulceration = 3/yr

Plus 2 of the following: Recurrent genital ulceration, Eye lesions, Skin lesions, Positive pathergy test

Question 6

What does aphthous ulceration present as in Behcet’s disease?

Answer 6

More aggressive
Major
Soft palate/ oropharynx involved
Multiple sites

Question 7

What is the first line treatment for mouth ulcers?

Answer 7

Topical tx.

Question 8

What is the first line treatment for mucocutaneous lesions?

Answer 8

Topical corticosteroids for oral and genital lesions

Colchicine (0.5-1.5mg/day) for oral/ genital/ skin lesions