ICHTHYOSIS CONGENITA GRAVIS OF FOETALIS
Dr. Atul A. Kulkarni, M.D. (Paed)*, Dr. Sachin M. Andhalkar, Resident**
Department of Paediatrics, Ashwini Sahakari Rugnalaya & Research Centre, Solapur.*, Department of Paediatrics, Ashwini Sahakari Rugnalaya & Research Centre, Solapur.**
Case History: 34 weeks male neonate delivered by primigravida who did not received any teratogenic drugs antenatally. Infant appears to be constricted in thick yellow-white skin armor shortly after birth, cracks up to a centimeter deep appears in the skin. Dark red cutis is visible at the base of clefts and face is altered, passed recognition with ectropion of mouth and eyes. The ears appears packed and in armor.

Hands and feet are also firmly constricted and the fingers and toes are immobilized. Baby did not survive.

Ichthyosis Congenita Gravis
It is an extreme lethal form with severe disorders of skin keratinisation, as the horny skin masses split in a rhomboidal pattern postpartum. Such children have also been called 'Harlequin foetus'. It is an autosomal recessive disorder.
  1. Frequency: 1: 3,00,000 in newborn
  2. Prognosis: Death in newborn period as a result of infection, sepsis and respiratory disorder.
  3. Treatment: Attempt to peel-off the horny plates in a humid incubator environment and use of oral retinoids and oil.
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