Author/Authors :
Meymandi, Simin S. Pathology and Stem Cell Research Center - Afzalipour Medical School - Kerman University of Medical Sciences, Kerman , Mehrolhasani, Niloofar Department of Dermatology - Afzalipour Medical School - Kerman University of Medical Sciences, Kerman , Ulmer, Marcie Department of Dermatology and Skin Science - University of British Colombia, Vancouver, Canada , Crawford, Richard I. Department of Dermatology and Skin Science - University of British Colombia, Vancouver, Canada
Abstract :
Harlequin ichthyosis is a rare and exceedingly severe form of
congenital ichthyosis with an incidence of approximately 1 in
300,000 births. These patients are at a high risk for neonatal
infection and septicemia. Most affected infants die within the
first days or weeks of life.
We report a male baby born with harlequin ichthyosis. There
is limited information regarding the course and prognosis of
neonates affected with harlequin ichthyosis. However, it is now
evident that these infants, depending on the severity, may have an
extended survival potential with intensive supportive measures
as well as the addition of retinoids.