ABSTRACT
INTRODUCTION: Peripheral T cell lymphomas are a heterogeneous group of post-thymic, mature lymphoid malignancies, accounting for approximately 10-15% of all non-Hodgkin's lymphomas. A rare entity within this group is represented by hepatosplenic T cell lymphoma, characterized by primary extranodal disease with infiltration of the liver and the spleen and by expression of the T cell receptor gamma delta chain. EXEGESIS: A 64-year old man with dermatomyositis developed rapid-onset paraparesia and deafness. Cerebrospinal fluid analysis revealed large granular lymphomatous cells with CD3+ CD4- CD8- CD7+ CD16- CD56- surface antigens, expressing the gamma delta T-cell receptor. There was no evidence of skin or bone marrow infiltration by lymphoma or any other involvement. This is the first report of dermatomyositis associated with a gamma delta T-cell lymphoma (GDTL). Moreover, primitive and isolated meningeal involvement of such lymphomas has never been described before. CONCLUSION: GDTL should be added to the differential list of neoplasia associated with dermatomyositis. Physiopathological mechanisms implicated in the neurological involvement of such lymphomas need to be elucidated.
Subject(s)
Dermatomyositis/etiology , Liver Neoplasms/diagnosis , Lymphoma, T-Cell/diagnosis , Paraneoplastic Syndromes/diagnosis , Splenic Neoplasms/diagnosis , Humans , Liver Neoplasms/metabolism , Lymphoma, T-Cell/metabolism , Male , Middle Aged , Receptors, Antigen, T-Cell, gamma-delta/metabolism , Splenic Neoplasms/metabolismABSTRACT
INTRODUCTION: Adult dermatomyositis is a rare inflammatory myopathy associated with typical cutaneous lesions and an increased incidence of internal malignancies, notably cancers of the female genital tract. Nevertheless, fallopian tube carcinoma is exceptionally associated with dermatomyositis. EXEGESIS: We report an unusual case of dermatomyositis because of cutaneous necrosis revealing a cancer of the fallopian tube. CONCLUSION: Predictive factors of cancer can improve prognosis of dermatomyositis due to earlier diagnosis of associated cancer. In our observation as in literature review, cutaneous necrosis lesions are highly predictive of an associated neoplasia even as rare as a fallopian tube carcinoma.