Delayed Onychodystrophy of Incontinentia Pigmenti: An Evidence-based Review of Epidemiology, Diagnosis and Management

Incontinentia pigmenti (IF) is an X-linked dominant disorder that is seen in females. Although the medical literature has focused strongly on the skin manifestations of IF, there is lack of comprehensive reviews focused on delayed nail lesions of IF. The authors thus tabulated reported nail changes in IF with a focus on diagnosis and management of this delayed nail presentations. This review emphasizes several observations with strong clinical relevance to management of IF. In particular, nail lesions may be additional clue to vague or late-presenting cases of IF. Nail lesions are most common on the first three digits of the hands, can affect multiple digits on multiple limbs, present from three to 45 years of age, and present on average over 20 years after skin lesions present. Clinically, nail lesions can be subtle and often are indicators of underlying tumor. Finally, nail lesions are delayed and destructive in nature, despite the clinically resolved cutaneous lesions, and require continual vigilance by pediatricians and dermatologist.