Incontinentia Pigmenti: A Case Report in a 1 Month Old Female Baby

Introduction: Incontinentia pigmenti, also known as Bloch- Sulzberger syndrome is a rare X linked dominant disorder that presents as skin lesions most commonly. This condition occurs due to mutation in NEMO (NF kB essential modulator) gene. It is a multisystemic disease that involves cutaneous tissues, dental, ocular and central nervous system besides other organs. Here, a case report in a 1 month old female baby is being discussed. Case Report: A female baby of 1 month presented to the dermatology OPD with features of hypertrophic, wart like lesion over the extremities (in a linear pattern), trunk and face. These lesions presented initially as vesicles and bullae during the first one week of life. These lesions followed blaschko?s line. The baby also has patches of alopecia seen over the scalp. Clinical diagnosis of Incontinentia pigmenti was made. Conclusion: Incontinentia Pigmenti is a multisystemic disease, so it is crucial to examine the patient with a multidisciplinary approach. All the systems should be carefully examined, regular follow up to pediatric ophthalmologist is critical. Radiological evaluation is crucial for any dental abnormalities. Dermatological management is aimed at reducing the risk of infection of blisters by giving antibiotics and maintain proper hygiene.