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Pediatr Infect Dis J 1997

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Sometimes pagetoid spread can be very marked and involve almost the entire lesion such melanocytic proliferation is generally referred to as a melanocytic acral nevus with intraepidermal ascent of cells (MANIAC) ( Fig

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Conditions for which this effect has been demonstrated are: glutaric aciduria II, methyl malonic aciduria, propionic acidemia, and medium chain fatty acylCoA dehydrogenase deficiency.7,8 Autointoxication occurs in these patients due to the accumulation of acylCoA compounds that disrupt intermediary metabolism

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