A 17-year old female patient was referred to our Department. Clinical examination revealed white, hyperkeratotic lesions on the dorsal part of the tongue, together with symmetric lesions on the buccal mucosa. The patient also showed dystrophic changes of the nails, both on hands and on feet, as well as bullous lesions on the soles. After detailed clinical and histologic examinations, diagnosis of pachyonychia congenita was made.
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