A case of angiocentric T-cell lymphoma with glabellar skin being the o
nly affected site was reported. A 43-year-old woman had a several mont
hs' history of glabellar swelling followed by progressive and destruct
ive ulceration of the region, Histopathology of the biopsied specimen
showed panniculitis with atypical lymphocytes and degenerative vessels
filled with atypical cells. Most of the infiltrated lymphocytes were
positive for CD2, CD3, CD4 and CD5. Antibodies to Epstein-Barr virus w
ere detected in the patient serum, Intensive chemotherapy using a prot
ocol of the L17M regimen was partially effective for clinical manifest
ations. Angiocentric T-cell lymphoma presenting as lethal midline gran
uloma is a rare but distinct entity with an acute fatal course.