It is a familial hereditary genodermatosis, of dominant character and starting during childhood or adolescence.  It appears symmetrically on the skin on the nasogenial sulci, scalp, retroauricular region, chest, dorsal and sacral region.  The buccal mucosa is the most affected with lesions that group as minute, white papules and whitish, flat plaques.  Grinspan cites them in his book as deep granules which raise the buccal mucosa and lips, the mucosa being sometimes corrugated

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