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The new classification of schwannomatoses is based on the coupling of the causative genes (i.e., NF2, SMARCB1 and LZTR1 ) and on the histological hallmark of these syndromes (i.e., schwannomas) [3]

Melanin deposited in the epidermis undergoes natural turnover as keratinocytes desquamate (shed), and without continued MC1R stimulation, melanocytes revert to baseline synthetic rates

In extreme cases patients die unless they get a liver transplant