A Case of Type 1 Pachyonychia Congenita (Jadassohn-Lewandowsky Syndrome).
- Author:
Ji Hoon SIM
1
;
Jung Hoon YANG
;
Sung Yul LEE
;
Moon Kyun CHO
;
You In BAE
;
Sanghoon LEE
Author Information
1. Department of Dermatology, College of Medicine, Soonchunhyang Universiy, Bucheon, Korea. shlee@schmc.ac.kr
- Publication Type:Case Report
- Keywords:
Pachyonychia congenita
- MeSH:
Adult;
Blister;
Extremities;
Heel;
Humans;
Keratoderma, Palmoplantar;
Keratosis;
Leukoplakia, Oral;
Molecular Biology;
Nails;
Pachyonychia Congenita
- From:Korean Journal of Dermatology
2011;49(3):265-268
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Pachyonychia congenita (PC) is characterized by hypertrophic nail dystrophy, focal palmoplantar keratoderma and blistering, oral leukokeratosis, cyst formation, palmoplantar hyperhydrosis, and follicular keratoses on the trunk and extremities. PC is diagnosed by clinical findings and molecular genetic studies. A 26-year-old man presented with hypertrophic nail dystrophy and subungual debris of all 20 nails, hyperkeratotic plaques on the heels of both soles, and oral leukokeratosis. He had no family history of similar clinical findings. The patient's clinical presentation and history were consistent with PC. Herein we report on a rare case of pachyonychia congenita with a review of literature.
