Table 1

 Clinical manifestations

Mucocutaneous ulceration (80%–100%)Oral aphthous ulcers,1 genital ulcers
Cutaneous (35%–65%)Pathergy,2 erythema nodosum, thrombophelibitis, acneiform skin eruption, palpable purpura
Ocular (50%–79%)Anterior uveitis, posterior uveitis, corneal ulceration, conjunctivitis, papillitis, retinal vasculitis
Musculoskeletal (30–50%)Non-erosive asymmetric oligoarthritis, pseudopodagra, sacroiliitis
Neurological (10%–30%)Aseptic meningitis, encephalitis, peripheral Neuropathy, cranial nerve palsies, seizures, cerebellar ataxia, pseudobulbar palsy, extra pyramidal signs
RenalFocal proliferative glomerulonephritis, diffuse proliferative glomerulonephritis, membranous glomerulonephritis
CardiovascularPericarditis, myocarditis, coronary arteritis, conduction defects, endocarditis, endomyocardial fibrosis, intracranial aneurysms3