A case of subungual tumors of incontinentia pigmenti: A rare manifestation and association with bipolar disease

Incontinentia pigmenti (IP), or Bloch-Sulzberger syndrome, is an X-linked dominant disorder with male lethality caused by mutations in the IKBKG gene (also known as IKK-γ or NEMO), which is essential for nuclear factor-κB (NF-κB) activation and protects cells against tumor necrosis factor-α–induced apoptosis.1 In addition to the 4 cutaneous stages of IP (vesiculo-bullous, verrucous, hyperpigmented, and atrophic/hypopigmented), IP is also associated with ectodermal abnormalities, such as pegged teeth, alopecia, and anodontia as well as neurologic and ocular abnormalities.