Ataxie-télangiectasie

Three children afflicted with ataxia-telangiectasia are briefly presented; the main characteristics and etiopathogenic factors are summarized. Cutaneous signs enumerate: telangiectasias on sun-exposed areas; pigmentary disturbances; atopic dermatitis; microbial, viral and mycotic infections, and progeria-like dystrophy. In these three children, the high number of café-au-lait spots and hypochromic macules appeared at first suggestive of neurofibromatosis or tuberous sclerosis.