Canadian Medical Guide > Diseases > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Abnormalities > Skin Abnormalities > Poikiloderma Congenitale Terms and Definitions

Poikiloderma Congenitale

Medical Definition:	An autosomal recessive syndrome occurring principally in females, characterized by the presence of reticulated, atrophic, hyperpigmented, telangiectatic cutaneous plaques, often accompanied by juvenile cataracts, saddle nose, congenital bone defects, disturbances in the growth of hair, nails, and teeth, and hypogonadism.
Guide Notes:	skin & other abnorm; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES

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