Lymphomatoid papulosis with a natural killer-cell phenotype.
Lymphomatoid papulosis (LyP) is defined as a recurrent self-healing papulonodular eruption with the histological features of a (CD30+) cutaneous T-cell lymphoma. The atypical cells usually have a CD3+/-, CD4+/-, CD8-, CD30+, CD56- T-cell phenotype. We report an unusual case of LyP, in which the atypical cells expressed a CD3-, CD4-, CD8-, CD30+, CD56+ phenotype. Detailed phenotypic and genotypic analysis confirmed that these cells had a natural killer (NK)-cell phenotype. Lymphomas with an NK-cell phenotype usually have a poor prognosis. However, the waxing and waning of papular lesions for more than 20 years and the excellent response to low-dose oral methotrexate in this patient suggest similar clinical behaviour to LyP cases with a T-cell phenotype.
Bekkenk
M W
MW
Department of Dermatology, Leiden University Medical Centre, Albinusdreef 2, 2300 RC Leiden, the Netherlands. m.w.bekkenk@lumc.nl
Kluin
P M
PM
Jansen
P M
PM
Meijer
C J
CJ
Willemze
R
R
D019002:CD56 Antigen / Q000235:genetics; D003879:Dermatologic Agents / Q000627:therapeutic use; D005838:Genotype; D006801:Humans; D017730:Ki-1 Antigen / Q000235:genetics; D007694:Killer Cells, Natural / Q000276:immunology*; D017731:Lymphomatoid Papulosis / Q000188:drug therapy / Q000276:immunology*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D010641:Phenotype; D011379:Prognosis; D016896:Treatment Outcome
D019002:CD56 Antigen; D003879:Dermatologic Agents; D017730:Ki-1 Antigen; D008727:Methotrexate