Introduction: Incontinentia pigmenti (IP) is a rare X-linked dominant disorder caused by loss-of-function mutations in the IKBKG (NEMO) gene. It presents shortly after birth and progresses through four cutaneous stages—vesicular, verrucous, hyperpigmented, and hypopigmented, distributed along Blaschko’s lines. Multiorgan involvement may include the hair, nails, teeth, eyes, and central nervous system. This case is presented due to its rarity and the diagnostic challenges it poses in neonates, where it may be mistaken for infections or other blistering disorders. Early recognition is crucial to initiate timely multidisciplinary care and prevent complications. This report contributes by highlighting local experience and raising global awareness of this condition. We report herein a case of incontinentia pigmenti in a 26-day-old female patient.

Case report: A 26-day-old Filipino female, born at 35 weeks of gestation to a 23-year-old primigravid mother with no history of miscarriage, presented with hyperpigmented, linear, and reticulated patches with desquamation on the trunk and extremities, noted earlier in the neonatal period. On examination, the mother also exhibited a few ill-defined, hypopigmented linear patches on the upper extremities and back. A diagnosis of incontinentia pigmenti was made based on clinical morphology, histopathology showing eosinophilic spongiosis consistent with the vesicular stage, and clinical improvement with topical steroids and supportive skin care.

Conclusion: This case emphasizes the importance of recognizing the characteristic cutaneous clues of incontinentia pigmenti in the neonatal period, ultimately improving outcomes for affected patients.

Dr. Joco Damasco is a second-year Dermatology resident at the University of the East Ramon Magsaysay Memorial Medical Center (UERMMC), Philippines. He obtained his Doctor of Medicine degree from the University of the East Ramon Magsaysay Memorial Inc. and is currently undergoing specialty training in medical, surgical, and pediatric dermatology. Throughout his residency, he has participated in clinical case discussions, academic conferences, and departmental research activities. His training emphasizes evidence-based practice, clinicopathologic correlation, and multidisciplinary collaboration in the diagnosis and management of dermatologic diseases. This case report reflects his commitment to recognizing rare dermatologic conditions and highlighting the importance of early diagnosis and appropriate management. He hopes that sharing uncommon cases will contribute to greater awareness among clinicians, facilitate earlier recognition of these disorders, and ultimately improve patient care. As a dermatology resident, he remains committed to lifelong learning, academic excellence, and providing compassionate, patient-centered care.