ABRAHAM KWADZO AHİAKPA, ELORM KWAKU KPOFO TETTEH, GERALD AMPROFİ, PRECİOUS AKOSUA RAPHAELSON, MAGDALENE DEDE APPİAH, DOROTHY SACKEY
Pediatric Academic Case Reports - 2025;4(3):58-62
Harlequin Ichthyosis (HI), the most severe form of congenital ichthyosis, has evolved in management, leading to improved outcomes. However, these outcomes may be impacted by resource availability. Based on published reports, we report, the first case of HI in Ghana, our management challenges and the potential benefit of short-course vitamin A as a potential adjunct therapy in resource-constrained settings. We report a male neonate delivered at 35weeks+3days gestation to a 20-year-old primiparous mother. Physical examination revealed generalized thick, grey scaly skin with diamond shapes interspersed with deep fissures, accompanied by facial and limb dysmorphic features. Supportive care was provided in the Neonatal Intensive Care Unit (NICU), including skin care, a 7-day course of low-dose oral vitamin A, and antibiotics. The infant was discharged in a stable state to parents upon a request of discharge against medical advice from parents. However, the infant died at home, of unclear cause, on day 12 of life. Systemic retinoids have considerably improved HI management outcomes in high resource settings. We propose that vitamin A could be a potential adjunct therapy in low-resource settings. This case lays a platform for more robust studies in the future to explore this finding.
