ABSTRACT
Neurocutaneous melanosis or neurocutaneous melanocytosis is a rare sporadic congenital disorder characterized by the presence of giant and/or multiple satellite congenital melanocytic nevi in the skin and benign melanocytic pigmentation of the leptomeninges. These two defining features were recognized more than a century ago. A third characteristic feature is proliferative nodules arising from giant nevi. The etiology is unknown, but neurocutaneous melanosis is considered a developmental disorder of melanocyte precursors from neural crest. The distinctive unique distribution of the congenital giant nevi that gives a "garment" appearance is also an expression of the neural crest. The neurological manifestations often appear in infancy.The special association of neurocutaneous melanosis with Dandy-Walker malformation complex may be explained by a common pathogenesis. Mortality in infancy and childhood is high.
Subject(s)
Dandy-Walker Syndrome/complications , Melanosis/diagnosis , Melanosis/therapy , Neurocutaneous Syndromes/diagnosis , Neurocutaneous Syndromes/therapy , Brain/pathology , History, 19th Century , Humans , Melanosis/complications , Melanosis/history , Neurocutaneous Syndromes/complications , Neurocutaneous Syndromes/historyABSTRACT
The phenomenon of industrial melanism is the preeminent example of natural selection in textbooks and the popular media. Much of its fame stems from a set of pioneering and apparently definitive investigations by H.B.D. 'Bernard' Kettlewell in the early 1950s. There is a marked contrast in how the phenomenon and Kettlewell's work on it are perceived by the public and scientists. Tensions between these two perceptions have recently led to calls for the removal of the example from textbooks, and indeed allegations that Kettlewell committed fraud. This article (part of the Science in the Industrial Revolution series) will show that these charges are baseless and stem from a fundamental misunderstanding of the nature of science as a process.
