Blaschko line analogies in the central nervous system: a hypothesis.
In X-chromosome-linked skin disorders the pattern of involvement follows Blaschko lines. Patterns of changes analogous to cutaneous Blaschko lines in different X-linked diseases existed in other organs. There is no commonly accepted analogy to Blaschko lines in the central nervous system (CNS). The objective of this study was to consider a hypothesis of the existence of Blaschko lines in the CNS in the example of incontinentia pigmenti (IP). Articles were analyzed in which brain imaging methods were used in IP patients with CNS anomalies. In IP patients with neurological signs brain lesions usually were localized and extended radially. Affected areas did not correspond to territories vascularized by any determined artery. Radially distributed brain lesions morphologically match the radial unit model of cortical development. It can be proposed that in IP in CNS Blaschko line analogies, similar to those in the skin, represent the trace of development of the clone of neurons arising from the cell marked with IKBKG mutation. The hypothesis of the existence of Blaschko line analogies in CNS is supported by radially distributed CNS image findings in IP, the radial unit model of CNS development, and the common embryonic origin of skin, CNS, and eyes.
School of Medicine, University of Belgrade, Dr Subotića 8, 11000 Belgrade, Serbia; Dermatovenerology Clinic, Clinical Center of Serbia, Deligradska 34, 11000 Belgrade, Serbia. Electronic address: dtrpinac@eunet.rs.
Snežana
S
Minić
Dušan
D
Trpinac
Miljana
M
Obradović
D002490:Central Nervous System / Q000196:embryology / Q000473:pathology*; D006801:Humans; D051550:I-kappa B Kinase / Q000235:genetics; D007184:Incontinentia Pigmenti / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008954:Models, Biological; D009030:Mosaicism; D009154:Mutation / Q000235:genetics; D014057:Tomography, X-Ray Computed
C491731:IKBKG protein, human; D051550:I-kappa B Kinase
CNS; CT; IKBKG; IP; MRI; NEMO; NF-κB; NF-κB essential modulator; VEGF; central nervous system; computed tomography; incontinentia pigmenti; inhibitor of kappa B kinase gamma; magnetic resonance imaging; nuclear factor-kappa B; vascular endothelial growth factor