Incontinentia Pigmenti (IP) is a neurocutaneous syndrome, with malformations of cortical development and neurodevelopmental delay in some patients. Neonates with IP may develop acute encephalopathy with multifocal ischemic brain lesions with a speckled pattern on diffusion-weighted magnetic resonance imaging (MRI). We observed a similar MRI pattern in 4 female patients with IP who presented with childhood acute encephalopathy syndromes. These patients, aged 9 days to 13 years old, had acute neonatal encephalitis, Influenza A virus related acute necrotizing encephalopathy (ANE) of childhood, Influenza B virus related acute encephalopathy with biphasic seizures and late restricted diffusion (AESD) and acute disseminated encephalitis (ADEM) with transverse myelitis (TM). These lesions could possibly reflect the white matter changes in IP patients with encephalopathy.

色素失禁症 (IP) 是一种神经皮肤综合征，某些患者会出现皮质发育畸形和神经发育迟缓。患有 IP 的新生儿可能会出现急性脑病，伴有多灶性脑缺血病变，弥散加权磁共振成像 (MRI) 显示斑点模式。我们在 4 名患有儿童急性脑病综合征的 IP 女性患者中观察到类似的 MRI 模式。这些患者年龄在 9 天至 13 岁之间，患有急性新生儿脑炎、A 型流感病毒相关的儿童急性坏死性脑病 (ANE)、B 型流感病毒相关的双相发作和迟发性扩散受限的急性脑病 (AESD) 和急性播散性脑炎（ ADEM) 与横贯性脊髓炎 (TM)。