case_images: PMC11774900_01
case_images: Overlapping skin lesions of erythema and blisters, verrucous hyperplasia, and pigmentation on the trunk (A), and verrucous hyperplasia on the arm (B) at 5 months of age. Hyperpigmentation on the trunk (C and D) at 11 months of age.
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case_images: PMC11774900_01_fped-12-1490816-g001.jpg
case_images: F1
case_images: This progression underscores the dynamic nature of the dermatological condition and its adherence to the unique pattern defined by Blaschko's lines (Figure 1).
case_images: MRI of brain ischemia. (A,B) Magnetic resonance diffusion-weighted imaging (DWI) showed scattered foci of restricted diffusion in the bilateral frontal parietal occipital lobes and bilateral temporal lobes, (C) T2-weighted magnetic resonance image (MRI) displayed periventricular white matter hypersignal at 5 months old. At 11 months old, repeated DWI (D,E) showed normal signal and (F) T2-weighted MRI displayed slightly hypersignal in bilateral semiovale centers.
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case_images: PMC11774900_01_fped-12-1490816-g002.jpg
case_images: F2
case_images: Magnetic resonance angiography (MRA) revealed the presence of anterior and right posterior communicating arteries, absence of the left posterior communicating artery, and tortuosity of the basilar arteries and intracranial segments of the bilateral vertebral arteries, all of which are considered developmental abnormalities (Figure 2).
case_images: Skin biopsy showing inflammatory stage lesions typical of incontinentia pigmentia (hematoxylin-eosin; (A) original magnification x100 and (B) original magnification x400). The white arrows indicate vacuolar degeneration, red arrows indicate dermal inflammatory infiltrate including lymphocytes; black arrows, chromophilic cells.
case_images: fped-12-1490816-g003.jpg
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case_images: F3
case_images: The skin biopsy revealed vacuolar liquefaction of the basal layer (of focal epidermis) accompanied by mild eosinophilic and lymphocytic infiltration in the superficial dermis, indicative of pigmentation (Figure 3).
case_images: PCR analysis of IKBKG gene by agarose gel electrophoresis indicated the deletion of exon in IKBKG of this patient. The samples from left to right were positive control, negative control and the case respectively in (A,B).
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case_images: F4
case_images: This was confirmed by polymerase chain reaction (PCR) analysis of IKBKG gene (Figure 4).
case_images: PMC11774900_02