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).

Eosinophilia

Increased count of eosinophils in the blood.


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PMID (PMCID)
29197078
 FEMALE Infant, Newborn
Cardiopulmonary anomalies in incontinentia pigmenti patients.
Onnis G, Diociaiuti A, Zangari P, D'Argenio P, Cancrini C, Iughetti L, El Hachem M.
Int J Dermatol. 2018;57(1):40-45.
Incontinentia pigmenti (IP) is a rare inherited genodermatosis that usually involves the skin, and also teeth, oral cavity, central nervous system, eyes, blood with eosinophilia, and rarely skeletal system, breast, heart, and lungs.
20204229
 FEMALE Infant, Newborn
[Neonate with linear rows of vesiculobullous lesions].
Fernandez MF, Samela PC, Bujan MM, Merediz J, Pierini AM.
Arch Argent Pediatr. 2010;108(1):e5-8.
Along with these lesions, the neonate had eosinophilia, which made us arrive to the clinical diagnosis of Incontinentia Pigmenti.
1628974
 FEMALE Infant, Newborn
[Onset of disseminated Bloch-Sulzberger incontinentia pigmenti].
Schaller J, Kock M, Goos M.
Hautarzt. 1992;43(6):383-5.
Eosinophilic spongiosis and peripheral blood eosinophilia are important symptoms in the diagnosis of atypical incontinentia pigmenti.
1628974
 FEMALE Infant, Newborn
[Onset of disseminated Bloch-Sulzberger incontinentia pigmenti].
Schaller J, Kock M, Goos M.
Hautarzt. 1992;43(6):383-5.
The peripheral blood eosinophilia and the development of typical lesions led to the diagnosis of incontinentia pigmenti.
1923976
 FEMALE Infant, Newborn
Localization of eosinophil granule major basic protein in incontinentia pigmenti.
Thyresson NH, Goldberg NC, Tye MJ, Leiferman KM.
Pediatr Dermatol. 1991;8(2):102-6.
The characteristic finding of extensive tissue eosinophilia in incontinentia pigmenti, as well as the new finding of extracellular deposition of an eosinophil granule protein in lesional tissue, suggests the involvement of eosinophils in the pathogenesis of the disease.
3676095
 FEMALE Infant
Reticulate hyperpigmentation distributed in a zosteriform fashion: a new clinical type of hyperpigmentation.
Iijima S, Naito Y, Naito S, Uyeno K.
Br J Dermatol. 1987;117(4):503-10.
Like a variant of incontinentia pigmenti (IP), all four cases showed eosinophilia.
7138112
 FEMALE Adult
Mitral annuloplasty in endomyocardial fibrosis: an alternative to valve replacement.
Wood AE, Boyle D, O'Hara MD, Cleland J.
Ann Thorac Surg. 1982;34(4):446-51.
An X-linked congenital dermatological condition, Bloch-Sulzberger syndrome (incontinentia pigmenti), associated with chronic eosinophilia, was also present.
7138112
 FEMALE Adult
Mitral annuloplasty in endomyocardial fibrosis: an alternative to valve replacement.
Wood AE, Boyle D, O'Hara MD, Cleland J.
Ann Thorac Surg. 1982;34(4):446-51.
An X-linked congenital dermatological condition, Bloch-Sulzberger syndrome (incontinentia pigmenti), associated with chronic eosinophilia, was also present.