Erythrokeratoderma variabilis-like ichthyosis in Chanarin-Dorfman syndrome.

  title={Erythrokeratoderma variabilis-like ichthyosis in Chanarin-Dorfman syndrome.},
  author={Ramon Maria Pujol and Montserrat Gilaberte and Agust{\'i} Toll and Lourdes Florensa and Josep Lloreta and Mar{\'i}a Antonia Gonz{\'a}lez-Ense{\~n}at and Judith Fischer and Antoni Az{\'o}n},
  journal={The British journal of dermatology},
  volume={153 4},
Neutral lipid storage disease (Chanarin-Dorfman syndrome) is an autosomal recessive metabolic disorder associated with congenital ichthyosis and a multisystemic accumulation of neutral lipids (lipid droplets) in various types of cells. The clinical presentation has been reported to correspond to that of nonbullous congenital ichthyosiform erythroderma. We report a 4-year-old boy presenting a generalized ichthyosiform disorder manifested by migrating scaly plaques alternating with areas of… CONTINUE READING


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