Rare, multisystem, chronic disease, characterized by oral and genital mucocutaneous ulcerations.
Associated symptoms: skin rashes, arthritis, thrombophlebitis, uveitis, colitis, and neurologic symptoms.

Epidimiology

Age of Onset: Third and fourth decades.
Sex: Males > females.
Prevalence: Highest in Japan (1:10,000), the Middle East (2:10,000). Rare in northern Europe, United States (1:100,000)

Signs & Symptoms

• Aphthous stomatitis
• Genital ulcers
• Dermal - papulovesicular, erythema nodosum, pathergy, erythema multiforme, vasculitis, pyoderma
• Ocular - iritis, iridocyclitis, chorioretinitis, hypopyon, hemorrhage, papilledema, optic atrophy
• Morning stiffness - in 1/3 patients
• Polyarthritis - self-limited and predominantly affecting lower extremities
• Thrombophlebitis - peripheral, pulmonary, cerebral, Budd Chiari syndrome
• Neurologic - cranial nerve palsy, hemiplegia, intracranial hypertension, meningomyelitis and recurrent meningitis, confusional state
• GI - aphthous ulcers, colitis, melena
• Pulmonary infiltrates - possibly related to thrombosis
• Myopathy/myositis - rare
• Peripheral gangrene - rare

Localized Therapy for Aphthous Ulcers

• Topical steroids
• Intralesional steroids
• Thalidomide
• Colchicine
• Dapsone

Systemic Therapy

• Prednisone with or without Chemotherapy