- Rare; Young F; Japan
- Vasculitis of aorta and aortic branches
- HTN, CHF, arrhythmias, stroke, seizures
- Constitutional symptoms during early phase
- Skin findings in 15-20%
- Erythema nodosum like nodules
- Erythema induratum
- Pyoderma gangrenosum-like lesions
- Granulomatous vasculitis