First symptoms appear between birth and 22 years
of age; prognosis, age of onset, and severity of the lesions are highly
variable: skin lesions: rash with diffuse telangiectasia, ecchymosis, difficult to cure
leg ulcers; characteristic facies: prominent skull sutures, ptosis and proptosis; recurrent infections of the skin,
chronic ear and sinus infections; impaired motor or cognitive development may be present. Treatment: oral
ascorbate or manganese is useful for the treatment of skin lesions.