NEONATAL INCONTINENTIA PIGMENTI – A CASE REPORT

Incontinentia pigmenti or Bloch-Sulzberger syndrome, is a rare multisystem, X-linked dominant disorder that most commonly occurs in female newborns. It is usually lethal, and most pregnancies with male fetuses result in miscarriage or stillbirth, while in female newborns, it can appear with different severity and multiorgan symptoms, including dermatological, neurological, ophthalmological, and dental abnormalities. Skin changes usually appear immediately after birth or in the first few weeks of life, following four stages: vesicular, verrucous, hyperpigmented, and hypopigmented. In the neonatal period, IP is clinically diagnosed based on the appearance of vesicles arranged in a linear pattern following Blaschko’s lines, representing the first stage of skin involvement. The differential diagnosis of a vesicular eruption in a neonate is extensive and includes various infectious and non-infectious causes. We report a case of a one-day-old female neonate who presented at birth with erythematous vesicles linearly distributed on the extremities, over the thorax and abdomen. Based on suspicious Blaschkoid skin lesions and eosinophilic skin infiltration in biopsy, incontinentia pigmenti was diagnosed. Early recognition of IP is crucial for appropriate management and monitoring of potential complications, particularly those involving the central nervous system and eyes. Although there is no specific cure for IP, a multidisciplinary approach involving dermatologists, neurologists, ophthalmologists, and dentists can help optimize the quality of life of affected infants.