A Case with Pachyonychia Congenita and B-cell Lymphoma
-
Vitorino Modesto dos Santos
,
Thiago Pereira Loures
,
João Daniel Bringel Rego
,
Christiane Aires Teixeira
,
Kayursula Dantas de Carvalho
,
Afonso Lucas Oliveira Nascimento
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.
Jadassohn J, Lewandowsky F. Pachyonychia congenita. Queratosis disseminata circumscripta (folicularis). Tylomata. Leucokeratosis linguae. In: Neisser A, Jacobi E, editors. Ikonographia Dermatologica. 1st ed. Berlin: Urbach and Schwarzemberg; 1906: p. 29-31.
Al Aboud A, Al Aboud K. Josef Jadassohn (1863-1936), Felix Lewandowsky (1879-1921), and their syndrome. Clin Cosmet Investig Dermatol 2011;4:179-82.
Ehsani A, Moeineddin F, Rajaee A. Pachyonychia congenita with woolly hair in a ten month old infant. Indian J Dermatol Venereol Leprol 2008;74(5):485-6.
Balasubramanian S, Kaarthigeyan K, Ramnath B. Pachyonychia congenita with unusual features. Indian Pediatr 2009;46(10):897-9.
Karen JK, Schaffer JV. Pachyonychia congenita associated with median rhomboid glossitis. Dermatol Online J 2007;13(1):21.
Kaspar RL, Leachman SA, McLean WH, et al. Toward a treatment for pachyonychia congenita: report on the 7th Annual International Pachyonychia Congenita Consortium meeting. J Invest Dermatol 2011;131(5):1011-4.
Nikoo A. Pachyonychia congenita-associated alopecia. Case Rep Pathol 2012;2012:850658.
Roche-Gamón E, Mahiques-Santos L, Vilata-Corell JJ. Paquioniquia congénita. Piel 2006;21(2):72-8.
Rodríguez H, Cuestas G, Zanetta A, et al. Pachyonychia congenita with involvement of the larynx. ActaOtorrinolaringol Esp 2013;6519(12):264-6.
Smith FJD, Hansen CD, Hull PR, et al. Pachyonychia Congenita. In: Pagon RA, Bird TD, Dolan CR, et al, editors. GeneReviews™. Seattle (WA): University ofWashington, Seattle; 1993-2006: p. 1-25.
Vaccaro M, Guarneri F, Barbuzza O, et al. Pachyonychia congenita tarda affecting only the nails. Dermatol Online J2008;14(2):12.
Dos Santos VM, Marques HV JR, Lima CDO C, et al. Yellow nail syndrome and adnexal tumour: causal or casual association? Indian J Chest Dis Allied Sci 2010;52(1):51-3.
Seve P, Thieblemont C, Dumontet C, et al. Skin lesions in malignancy. Case 3. Yellow nail syndrome in non- Hodgkin's lymphoma. J Clin Oncol 2001;19(7):2100-1.
Menon BS, Ibrahim SB, Juraida E, et al. Pachyonychia congenita. J Paediatr Child Health 2008;44(6):386.
| Files | ||
| Issue | Vol 52, No 7 (2014) | |
| Section | Original Article(s) | |
| Keywords | ||
| Jadassohn-Lewandowsky syndrome B-cell lymphoma Pachyonychia congenita Palmoplantar keratoderma | ||
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