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Most newborns that have the neurofibromatosis gene mutation show few or no signs. Cafe–au–lait spots are usually noticed in the first few weeks of life, or may appear earlier.. Neurofibromas are not often found in infancy. One exception is the plexiform neurofibroma. Café–au–lait spots (pigmented macules resembling 'birthmarks') are the hallmark of. Café–au–lait spots (Figure 121-1) are usually the first feature to appear; some may be. .. The presence of only café au lait spots

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