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Published on: March 30, 2018
A case with pachyonychia congenita and B-cell lymphoma
Vitorino Modesto dos Santos1, Thiago Pereira Loures2, João Daniel Bringel Rego3
1Catholic University Medical Course, Brasília-DF, Brazil . AND Department of Internal Medicine, Armed Forces Hospital, Brasília-DF, Brazil. vitorinomodesto@gmail.com.
Abstract:
Pachyonychia congenital (PC) is a rare autosomal dominant genodermatosis characterized hyperkeratosis affecting the nails and palmoplantar areas, oral leukokeratosis, and cystic lesions. A 39-year-old woman with PC type 1 (Jadassohn-Lewandowsky syndrome) and B-cell lymphoma is described. No similar disorders or parental consanguinity were found in her family. Typical features of PC developed since her early childhood and the diagnosis of B-cell lymphoma was established seven years ago, without a clear causal relation between these entities. Despite inherent limitations of a single case, this report may contribute to PC understanding.
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