KMID : 0363220110490030265
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Korean Journal of Dermatology 2011 Volume.49 No. 3 p.265 ~ p.268
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A Case of Type 1 Pachyonychia Congenita (Jadassohn-Lewandowsky Syndrome)
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Sim Ji-Hoon
Yang Jung-Hoon Lee Sung-Yul Cho Moon-Kyun Bae You-In Lee Sang-Hoon
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Abstract
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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.
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KEYWORD
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Pachyonychia congenita
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