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Patients develop poorly demarcated, diffuse, painless white plaques on the oral mucosa, usually the buccal mucosa and tongue, usually within the first two decades of life.21,54 Other conditions that may appear similar clinically include hereditary benign intraepithelial dyskeratosis, another rare mucosal disorder that occurs in a racial isolate in South Carolina,5557 and pachyonychia congenita.5860 Oral lesions of dyskeratosis congenita are true leukoplakias occurring in approximately 65% of patients and are usually dysplastic.61,62 Biopsy or exfoliative cytology is always indicated and shows perinuclear eosinophilic condensations (representing abnormal keratin aggregation) and distinguishes it from other mucosal disorders discussed below