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Paper Information

Journal:   ACTA MEDICA IRANICA   2014 , Volume 52 , Number 7; Page(s) 578 To 581.
 
Paper: 

A CASE WITH PACHYONYCHIA CONGENITA AND B-CELL LYMPHOMA (CASE REPORT)

 
 
Author(s):  DOS SANTOS VITORINO MODESTO*, LOURES THIAGO PEREIRA, BRINGEL REGO JOAO DANIEL, TEIXEIRA CHRISTIANE AIRES, DE CARVALHO KAYURSULA DANTAS, OLIVEIRA NASCIMENTO AFONSO LUCAS
 
* CATHOLIC UNIVERSITY MEDICAL COURSE AND ARMED FORCES HOSPITAL, BRASILIA-DF, BRAZIL
 
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-yearold 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.

 
Keyword(s): JADASSOHN-LEWANDOWSKY SYNDROME, B-CELL LYMPHOMA, PACHYONYCHIA CONGENITA, PALMOPLANTAR KERATODERMA
 
References: 
 
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