title: A skin lesion that catches the eye.
abstract: Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) is rare and only represents 1% of all cutaneous T-cell lymphomas. To our knowledge, only 40 cases have been described. It often presents with generalised skin lesions, preferentially affecting the extremities. There is a well-documented association with haemophagocytic syndrome. Treatment is difficult since PCGD-TCL is often resistant to chemotherapy and radiotherapy. Most case reports describe an aggressive clinical course with an estimated mean survival of 15 months. We present a 72-year-old female patient with stage IV primary cutaneous gamma-delta T-cell lymphoma. Our patient presented with fever, night sweats and multiple skin lesions (figure 1). Computed axial tomography of chest and abdomen revealed multiple solid nodular lesions in both kidneys. During admission a subconjunctival lesion appeared and progressed rapidly (figure 2). Histopathological examination of skin biopsy revealed infiltration of atypical lymphocytes with hyperchromatic irregular nuclei. Immunophenotyping pattern of skin biopsy was compatible with PCGD-TLC. Clonal gamma-delta T-cells were also detected by immunohistochemical analysis of peripheral blood and bone marrow. Polymerase chain reaction amplification revealed clonal rearrangement of the T-cell receptor gamma chain gene. These findings together were consistent with stage IV primary cutaneous gamma-delta T-cell lymphoma. The rapid progression of the subconjunctival extra-nodal manifestation is characteristic for the aggressive course of this lymphoma. Our patient was treated with two cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone). However, her clinical condition deteriorated rapidly. She declined further therapy and died within three months of initial presentation.
authors.affiliation: Department of Internal Medicine, IJsselland Hospital, Capelle aan den IJssel, the Netherlands.
authors.forename: L
authors.initials: L
authors.lastname: Louter
authors.forename: I P G
authors.initials: IP
authors.lastname: Botden
authors.forename: R F J
authors.initials: RF
authors.lastname: Schop
mesh_terms: D000368:Aged; D005134:Eye Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis* / Q000235:genetics; D012878:Skin Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*
chemical_list: D016692:Receptors, Antigen, T-Cell, gamma-delta