Erythroderma and non-Hodgkin T-cell lymphoma: what else, apart from Mycosis Fungoides and Sézary syndrome?
Alessandro Pileri, Cinzia Pellegrini, Claudio Agostinelli, Vieri Grandi, Annalisa Patrizi, Pier Luigi Zinzani, Nicola Pimpinelli
Abstract
Alessandro Pileri, Cinzia Pellegrini, Claudio Agostinelli, Vieri Grandi, Annalisa Patrizi, Pier Luigi Zinzani, Nicola Pimpinelli
Abstract
BACKGROUND: Peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS), are a rare condition characterised by specific histology, nodal presentation, and a poor prognosis. In total, 10-18% of patients present with cutaneous involvement which is regarded as a poor prognostic marker. However, cutaneous PTCL-NOS lesions have been rarely reported in the literature. OBJECTIVES: We sought to describe PTCL-NOS cases characterised by erythrodermic dissemination to the skin. MATERIALS & METHODS: Three cases of PTCL-NOS were investigated; all male, with a mean and median age of 55 and 51 years, respectively. RESULTS: All patients underwent aggressive chemotherapeutic protocols with only transient improvement of the disease, and died within two years of follow-up. CONCLUSION: Dermatologists should be reminded that erythroderma and lymph node enlargement do not represent an exclusive paradigm for erythrodermic cutaneous T-cell lymphoma, and that these features can be due to a systemic lymphoma that should be considered in the differential diagnosis.
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BACKGROUND: Peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS), are a rare condition characterised by specific histology, nodal presentation, and a poor prognosis. In total, 10-18% of patients present with cutaneous involvement which is regarded as a poor prognostic marker. However, cutaneous PTCL-NOS lesions have been rarely reported in the literature. OBJECTIVES: We sought to describe PTCL-NOS cases characterised by erythrodermic dissemination to the skin. MATERIALS & METHODS: Three cases of PTCL-NOS were investigated; all male, with a mean and median age of 55 and 51 years, respectively. RESULTS: All patients underwent aggressive chemotherapeutic protocols with only transient improvement of the disease, and died within two years of follow-up. CONCLUSION: Dermatologists should be reminded that erythroderma and lymph node enlargement do not represent an exclusive paradigm for erythrodermic cutaneous T-cell lymphoma, and that these features can be due to a systemic lymphoma that should be considered in the differential diagnosis.
Key concepts: Mycosis fungoides, Erythroderma, Medicine, Dermatology, Lymphoma, Peripheral T-cell lymphoma, Pathology, T cell