ABSTRACT
Primary cutaneous aggressive epidermotropic cytotoxic CD8 positive T cell lymphoma, is an uncommon disease, with an aggressive clinical behavior. Differentiation with other types of cutaneous T-cell lymphoma (CTCL) that express a CD8+ cells, is based only on clinical grounds and in certain morphological characteristics, such as a marked epidermotropism with squamous cell necrosis. We report a 50-year-old male presenting with painless cutaneous lesions appearing in trunk, limbs, scalp and face, suggestive of cutaneous lymphoma. He was admitted to the hospital in bad conditions, with confluent papules and tumors, some of them ulcerated and with foul smelling honey-colored crusts, involving the complete body surface. Cutaneous biopsy demonstrated a CD8 positive epidermotropic cytotoxic T cell lymphoma. He was treated with chemotherapy with an excellent initial response, but cutaneous lesions reappeared after four cycles. He did not respond to rescue chemotherapy and died seven months after diagnosis.
Subject(s)
Humans , Male , Middle Aged , Lymphoma, T-Cell, Cutaneous/pathology , Skin Neoplasms/pathology , T-Lymphocytes, Cytotoxic/pathology , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy , Cyclophosphamide/administration & dosage , Doxorubicin/administration & dosage , Fatal Outcome , Lymphoma, T-Cell, Cutaneous/drug therapy , Prednisone/administration & dosage , Skin Neoplasms/drug therapy , Vincristine/administration & dosageABSTRACT
We report a 53-year-old female on chronic dialysis, presenting with weight loss, the development of big soft masses in the shoulders ("shoulder pads"), forearms and buttocks, macroglossia and rigidity of hands and lower limbs. Laboratory confirmed the presence of amyloidosis and myeloma with lambda chain restricted plasma cell infiltration of bone marrow. The diagnosis of multiple myeloma associated with primary amyloidosis in a patient with terminal renal failure, was reached.