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CEP is a severe blistering porphyria with multi-system involvement.

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Separating this complex and potentially deadly “internal” gut content from our circulation and other organ systems, including the brain, is a tightly regulated interface consisting of a mucus layer, a single epithelial layer held together by tight junction proteins (e.g., zonulin and occludin), immunoglobulin A, defensins, and a carefully orchestrated immune surveillance in the lamina propria of the gut [136]