{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T07:38:44Z","timestamp":1791272324696,"version":"4.1.0"},"reference-count":153,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2004,12,15]],"date-time":"2004-12-15T00:00:00Z","timestamp":1103068800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pigment Cell Research"],"published-print":{"date-parts":[[2005,2]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disorder, affecting approximately 1 in 3500 individuals. The most commonly seen tumors in NF1 patients are the (sub)cutaneous neurofibromas. However, individuals with NF1 typically present in childhood with well\u2010defined pigmentary defects, including caf\u00e9\u2010au\u2010lait macules (CALMs), intertriginous freckling and iris Lisch nodules. NF1 is considered a neurocristopathy, primarily affecting tissues derived from the neural crest. Since the pigment producing melanocyte originates in the neural crest, the presence of (hyper)pigmentary lesions in the NF1 phenotype because of changes in melanocyte cell growth and differentiation is to be expected. We want to discuss the pigmentary cutaneous manifestations of NF1 represented by CALMs and intertriginous freckles and the pigmentary non\u2010cutaneous manifestations represented by iris Lisch nodules. Several hypotheses have been suggested in explaining the poorly understood etiopathogenesis of CALMs. Whether other pigmentary manifestations might share similar etiopathogenic mechanisms remains obscure. Additional attention will be drawn to a readily seen phenomenon in NF1: hyperpigmentation overlying (plexiform) neurofibromas, which could suggest common etiopathogenetic\u2010environmental cues or mechanisms underlying CALMs and neurofibromas. Finally, we want to address the relationship between malignant melanoma and NF1.<\/jats:p>","DOI":"10.1111\/j.1600-0749.2004.00206.x","type":"journal-article","created":{"date-parts":[[2005,1,12]],"date-time":"2005-01-12T14:16:14Z","timestamp":1105539374000},"page":"13-24","source":"Crossref","is-referenced-by-count":65,"title":["Pigment cell\u2010related manifestations in neurofibromatosis type 1: an overview"],"prefix":"10.1111","volume":"18","author":[{"given":"Sofie 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