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Pachyonychia Congenita Nature of disease

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A rare autosomal dominant condition caused by mutations in the K6a, K16, and K17 keratins that mostly affect nail beds and to a lesser extent the oral mucosa. Pachyonychia congenita is seen within two syndromes: Jadassohn-Lewandowsky syndrome and Jackson-Lawler syndrome.

Oral Pathology in Clinical Dental Practice

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