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.
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Age
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Skin Lesions
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Stage
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onset soon after birth
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erythema, vesicles or blisters/bullae, pustules; occur on the extremities and circumferentially around the trunk
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1
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2 to 6 weeks after birth, lasting up to 6 to 12 months of age
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papules, verrucous or warty lesions, hyperkeratosis; may be linearly arranged on the distal extremities
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2
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from 12 - 26 weeks after birth to late adolescence or early adulthood
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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
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3
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late adolescence to adulthood
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pallor, atrophy, scarring, alopecia, hypopigmentation; especially in streaks on the lower extremities
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4
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