What is it about?
Introduction: Pachyonychia congenita (PC) is a very rare genetic condition triggered by mutations in one of five genes that encode for keratin. It is usually characterized by nail dystrophy and painful palmoplantar keratomas, as well as oral leukokeratosis, natal/prenatal teeth, and sebaceous cysts. This case details PC in an adult, to include the management of the patient and clinicopathologic characteristics of the disorder.
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Why is it important?
Case Description: A 78-year-old male patient with a known history of pachyonychia congenita presented with clinical findings of xerostomia, coated tongue, abnormal bilateral white lesions, and hyper-keratinization on the lateral borders of the tongue and buccal mucosa.
Perspectives
This case documents a rare genetic disorder which can lead to multiple systemic and oral health problems, and includes the management of the patient and clinicopathologic characteristics of the disorder.
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This page is a summary of: Pachyonychia Congenita: Clinical and Pathological Case Report, Journal of Dentistry and Oral Epidemiology, February 2026, Acquire Publications LLC,
DOI: 10.54289/jdoe2600101.
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