(1) Major criteria

Skin & mucosa
Yellowish papular lesions, confined to flexural areas of the body
Plaques of papules
Plaques of dot-like depressions resembling orange peel
Increased skin laxity; increased skin folding
Yellow reticular pattern on inner lip mucosa
Peau d'orange
Angioid streaks
Comets and comet tails
Complications with subchoroidal neovascularsiation, haemorrhage and macular degeneration; decreased or absent central vision

(2) Minor criteria

Optic head drusen
Cardio- and cerebrovascular
Accelerated atherosclerosis
Coronary artery disease
Angina pectoris
Intermittent claudication
Absent peripheral pulses
Mitral valve prolapse
Gastrointestinal hemorrhage
Calcifications in kidney, liver, spleen, pancreas with normal calcium/phosphorus balance
Testicular microlithiasis