A child with the rare Harlequin ichthyosis disease has been admitted to the Barishal Sher-e-Bangla Medical College Hospital (SBMCH).

Dr Bakir Hossain, director, SBMCH, said that the child, born at Char Shefali, under Mehendiganj upazila of Barishal, was admitted to the hospital’s neo-natal ward on Thursday afternoon.

Prof Dr Syed Zahid Hossain, former head of neo-natal and paediatric department of SBMCH, said ichthyosis is a genetically inherited ailment traced to the ABCA12 gene.

Individuals must inherit two recessive genes in order to show the disease—one from each parent, but the parents (“carrier”) show no signs of ichthyosis.  

Harlequin is an extremely rare form of ichthyosis and the most severe type. The newborn Harlequin child is covered with plates of thick skin that crack and split apart and need to be taken care of immediately for the first several weeks of the baby’s life at the neonatal intensive care unit, the pediatric expert said. The surviving children display dry, reddened skin, which may be covered with large thin scales, and sparse hair.

Physical development may be delayed by the enormous calories needed for their skin function, but mental and intellectual developments are expected to be normal.

 Antibiotic treatment may be necessary to prevent infection. Administration of oral etretinate (1 mg./kg of body weight) may accelerate the shedding of the thick scales.

Harlequin ichthyosis demands a meticulous skin care regimen to keep the skin moisturized and pliable and to prevent cracking and fissuring that may lead to infection, Prof. Dr Zahid added.