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Exceptionally, some patients with definite diagnosis of NF1 do not have CaLS, have an insufficient number of them, or the lesions have an atypical morphology.11,14 However, most patients with more than 6 CaLS without other markers of NF1 will end up developing NF1, usually before they are 5 years old, and almost always before they are 8 years old.14,15 The more typical the morphology of CaLS, the greater the probability that NF1 is confirmed, and there is no association between the number of CaLS and the severity of NF1.14 Histologically, CaLS have increased melanin in melanocytes and basal keratinocytes, but no melanocyte proliferation.16 The presence of macromelanosomes has also been observed, along with a greater concentration of melanin in CaLS of patients with NF1 than in sporadic CaLS.17,18 From the molecular point of view, it has been demonstrated that melanocytes of CaLS have a second NF1 mutation in the gene on the healthy allele.19 Small children with CaLS and NF1 are often referred to our clinics
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