Incontinentia pigmenti (IP) is a rare X-linked dominant genetic disorder affecting the skin, hair, teeth, and central nervous system. It is characterized by distinctive skin lesions that evolve through four stages: vesicular, verrucous, hyperpigmented, and atrophic. This clinical vignette on incontinentia pigmenti aims to guide residents in identifying and managing this uncommon condition. Through this case, users will learn about the clinical features of IP, its differential diagnosis, and appropriate diagnostic and management strategies.

This vignette is an excellent resource for residents preparing for OSCE-style examinations and can also be used as a self-directed learning tool for healthcare professionals seeking to enhance their knowledge and skills in managing incontinentia pigmenti.

We invite you to explore the associated learning module and quizzes to reinforce your knowledge and test your understanding of this important topic.

1. Levy, ML. (2020). Incontinentia pigmenti. UpToDate. Retrieved November 20, 2021, from https://www.uptodate.com/contents/incontinentia-pigmenti
2. Moss C., Browne F. (2018). Mosaicism and Linear Lesions. Bolognia, J.L., Schaffer, J.V., Cerroni, L (Eds.). Dermatology (4th ed.) (pp 1004-1025). Elsevier.