Cutaneous disease with onset from first months of life to the seventh decade.
20-30% of cases limited to the skin and associated with a normal life expectancy.
in majority of cases the disease is a multisystem process which is lethal.
Most common cause of death is microinfarctions in the intestines associated with bowel perforation and peritonitis.
Central nervous system frequently involved with vascular lesions.
Skin lesions consist of crops of papules on the trunk and extremities, sparing the face palms, soles and scalp.
Eye lesions may involve conjunctiva, sclera, retina, choroid and optic nerve.
Skin lesions may umbilicate and have central necrosis
Skin lesions generally precede visceral symptoms.