Author/Authors :
Dos Santos، Vitorino Modesto نويسنده Catholic University Medical Course, Bras?lia-DF, Brazil . AND Department of Internal Medicine, Armed Forces Hospital, Bras?lia-DF, Brazil. Dos Santos, Vitorino Modesto , Loures، Thiago Pereira نويسنده Department of Internal Medicine, Armed Forces Hospital, Bras?lia-DF, Brazil. Loures, Thiago Pereira , Rego، Jo?o Daniel Bringel نويسنده Division of Pneumology, Armed Forces Hospital, Bras?lia-DF, Brazil. Rego, Jo?o Daniel Bringel , Teixeira، Christiane Aires نويسنده Division of Pneumology, Armed Forces Hospital, Bras?lia-DF, Brazil. Teixeira, Christiane Aires , De Carvalho، Kayursula Dantas نويسنده Department of Internal Medicine, Armed Forces Hospital, Bras?lia-DF, Brazil. De Carvalho, Kayursula Dantas , Nascimento، Afonso Lucas Oliveira نويسنده Department of Internal Medicine, Armed Forces Hospital, Bras?lia-DF, Brazil. Nascimento, Afonso Lucas Oliveira
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.
