The cutaneous lesions in incontinentia pigmenti (IP) progresses through 4 clinical stages as the patient ages.


Features of the skin lesions:

(1) Not all patients show the skin lesions or the lesions may be overlooked.

(2) Not all stages occur in a patient.

(3) Some stages may overlap.


Skin Lesions


onset soon after birth

erythema, vesicles or blisters/bullae, pustules; occur on the extremities and circumferentially around the trunk


2 to 6 weeks after birth, lasting up to 6 to 12 months of age

papules, verrucous or warty lesions, hyperkeratosis; may be linearly arranged on the distal extremities


from 12 - 26 weeks after birth to late adolescence or early adulthood

hyperpigmented macules on the trunk that follows Blaschko's lines and on the extremities; these tend to fade over time but may persist in the groin


late adolescence to adulthood

pallor, atrophy, scarring, alopecia, hypopigmentation; especially in streaks on the lower extremities



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