Incontinentia pigmenti

Incontinentia pigmenti (IP) is a rare X-linked dominant multi-systemic ectodermal dysplasia usually lethal in males and presenting neonatally in females with a bullous rash along Blashko's lines (BL) followed by verrucous plaques evolving over time to hyperpigmented swirling patterns. It is further characterized by teeth abnormalities, alopecia, nail dystrophy and affects occasionally the retina and the central nervous system (CNS).

Epidermal nevus

Epidermal naevi are due to an overgrowth of the epidermis and may be present at birth (50%) or develop during childhood.


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PMID (PMCID)
24119172
 FEMALE
A case of linear porokeratosis treated with photodynamic therapy with confocal microscopy surveillance.
Curkova AK, Hegyi J, Kozub P, Szep Z, D'Erme AM, Simaljakova M.
Dermatol Ther. 2014;27(3):144-7.
Differential diagnosis includes linear lichen planus, lichen striatus, linear verrucous epidermal nevus, incontinentia pigmenti and linear Darier's disease.
11280469
 FEMALE
Linear and whorled nevoid hypermelanosis.
Mendiratta V, Sharma RC, Arya L, Sardana K.
J Dermatol. 2001;28(1):58-9.
The disorder may resemble incontinentia pigmenti, epidermal nevus, or zebra-like hyperpigmentation clinically.
8346111
 MALE
Linear and whorled nevoid hypermelanosis.
Alvarez J, Peteiro C, Toribio J.
Pediatr Dermatol. 1993;10(2):156-8.
This entity represents a disorder separated recently from other pigmentary alterations that follow Blaschko lines, such as Bloch-Sulzberger incontinentia pigmenti, hypomelanosis of Ito, early systematized epidermal nevus, and chimerism.
3204178
 MIXED_SAMPLE Infant
Linear and whorled nevoid hypermelanosis.
Kalter DC, Griffiths WA, Atherton DJ.
J Am Acad Dermatol. 1988;19(6):1037-44.
This condition must be differentiated from incontinentia pigmenti, early systematized epidermal nevus, extensive hypomelanosis of Ito, and chimerism.
4070090
 MIXED_SAMPLE Infant
Histologic changes resembling the verrucous phase of incontinentia pigmenti within epidermal nevi: report of two cases.
Fletcher V, Williams ML, Lane AT.
Pediatr Dermatol. 1985;3(1):69-74.
Histologic changes resembling the verrucous phase of incontinentia pigmenti within epidermal nevi: report of two cases.