E. Poszepczynska et al., Erythrodermic cutaneous T-cell lymphoma with disseminated pustulosis. Production of high levels of interleukin-8 by tumour cells, BR J DERM, 144(5), 2001, pp. 1073-1079
Interleukin (IL) -8 is a neutrophil chemoattractant cytokine with proinflam
matory and growth-promoting activities, which is involved in the pathogenes
is of several inflammatory diseases. It is found in high amounts in lesiona
l biopsies of pustular diseases such as psoriasis and palmoplantar pustulos
is. We report a 50-year-old woman with a 10-year history of erythroderma wi
th disseminated pustulosis. Skin biopsies showed an epidermotropic infiltra
te composed of atypical CD4+ CD8+ lymphocytes with numerous admixed neutrop
hils. Peripheral blood flow cytometric analysis revealed a major clonal sub
set of CD3+ CD4+ CD8+ T-cell receptor V beta 22+ atypical lymphocytes. Bone
marrow biopsy, lymph node biopsy and computed thoracoabdominal tomography
were normal. Serologies for human T-cell lymphotropic virus type I and huma
n immunodeficiency virus were negative. Our patient's status deteriorated d
espite topical (nitrogen mustard, psoralen plus ultraviolet A) and systemic
(interferon, methotrexate, multiagent chemotherapy) treatments, and she fi
nally died. We showed that our patient's peripheral blood lymphocytes (PBL)
spontaneously produced high amounts of IL-8. In contrast, PBL of patients
with classical Sezary syndrome produced lower amounts of IL-8. The producti
on of IL-8 by tumour T cells could explain this unusual clinical and histop
athological presentation of cutaneous T-cell lymphoma as disseminated pustu
losis.