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Journal of the American Academy of Dermatology
Volume 62, n° 3
pages 496-501 (mars 2010)
Doi : 10.1016/j.jaad.2009.03.001
Case Reports

Leg ulcer and thigh telangiectasia associated with natural killer cell CD56 large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome

Ana Filipa Duarte, MD a, d, , Ana Nogueira, MD a, Alberto Mota, MD, PhD a, d, Teresa Baudrier, MD a, Áurea Canelhas, MD b, Jorge Cancela, MD c, Margarida Lima, MD, PhD e, Filomena Azevedo, MD a
a Department of Dermatology and Venereology, Hospital São João, Porto, Portugal 
b Department of Pathology, Hospital São João, Porto, Portugal 
c Department of Hematology, Hospital São João, Porto, Portugal 
d Faculty of Medicine of Oporto, Porto, Portugal 
e Department of Hematology, Laboratory of Cytometry, Centro Hospitalar do Porto, Hospital Geral de Santo António, Porto, Portugal 

Reprint requests: Ana Filipa Duarte, MD, Department of Dermatology and Venereology, Hospital São João, Alameda Professor Hernâni Monteiro, 4200-319 Porto, Portugal.

Clonal disorders of large granular lymphocytes (LGL) represent a rare spectrum of biologically distinct lymphoproliferative diseases originating either from mature T cells or natural killer cells. Both subtypes can manifest as indolent or aggressive disorders. We report a 77-year-old woman with rheumatoid arthritis, splenomegaly, and neutropenia who developed a painful leg ulcer refractory to treatment and thigh telangiectatic lesions. Because of the association of rheumatoid arthritis, splenomegaly, and nonspecific neutropenia, the diagnosis of Felty syndrome was initially made. Further investigation allowed the diagnosis of a CD56 natural killer–cell LGL leukemia and documented skin infiltration by natural killer cells. Cutaneous manifestations of LGL leukemia have been rarely reported. This report of pseudo-Felty syndrome with CD56 LGL leukemia, presenting with a leg ulcer and telangiectasia, enhances the role of dermatology in the diagnosis of hematologic neoplasia.

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Key words : CD56 cells, large granular lymphocyte leukemia, leg ulcer, natural killer cells, pseudo-Felty syndrome, telangiectasia

Abbreviations used : EBV, LGL, NK, RA

 Funding sources: None.
 Conflicts of interest: None declared.

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