2019Journal of Clinical SciencesOpen access

Sezary syndrome in a 65-year-old schizophrenic patient

Abdullahi Umar, TahirTuraki Mohammed, MaryamShehu Ahmed, ModupeoplaOmotara A. Samaila, Hadiza Sani

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Abstract

Sezary syndrome (SS) is the leukemic variant of cutaneous T-cell lymphoma characterized by a triad of erythroderma, peripheral lymphadenopathy, and the presence of circulating atypical lymphoid cells (Sezary cells) in the blood. We present a 65-year-old female with a recent-onset schizophrenia, presenting with a 3-year history of recurrent generalized pruritus, erythroderma, and a 2-month history of recent-onset, mushroom-like skin tumors. Examination revealed generalized erythroderma affecting more than 80% of total body surface area and significant peripheral lymphadenopathy. Peripheral blood buffy coat examination showed Sezary cells which constituted more than 18% of total circulating lymphoid cells, and histology report of the wedge biopsy of skin tumor confirmed mycosis fungoides. The patient was commenced on cytotoxic chemotherapy with some improvement in symptoms. However, she relapsed 2 months later, with development of new fungating tumors and died from overwhelming sepsis. We report this case to highlight the occurrence of two comorbidities together, each of which may negatively worsen the progression of the other, and to report a case of SS as one of the rare causes of generalized exfoliative dermatitis. Thus, there is a need for early skin biopsy and histology in any elderly patient presenting with recurrent generalized pruritus and erythroderma.

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What this paper is about

Sezary syndrome (SS) is the leukemic variant of cutaneous T-cell lymphoma characterized by a triad of erythroderma, peripheral lymphadenopathy, and the presence of circulating atypical lymphoid cells (Sezary cells) in the blood. We present a 65-year-old female with a recent-onset schizophrenia, presenting with a 3-year history of recurrent generalized pruritus, erythroderma, and a 2-month history of recent-onset, mushroom-like skin tumors. Examination revealed generalized erythroderma affecting more than 80% of total body surface area and significant peripheral lymphadenopathy. Peripheral blood buffy coat examination showed Sezary cells which constituted more than 18% of total circulating lymphoid cells, and histology report of the wedge biopsy of skin tumor confirmed mycosis fungoides. The patient was commenced on cytotoxic chemotherapy with some improvement in symptoms. However, she relapsed 2 months later, with development of new fungating tumors and died from overwhelming sepsis. We report this case to highlight the occurrence of two comorbidities together, each of which may negatively worsen the progression of the other, and to report a case of SS as one of the rare causes of generalized exfoliative dermatitis. Thus, there is a need for early skin biopsy and histology in any elderly patient presenting with recurrent generalized pruritus and erythroderma.

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Available abstract

Sezary syndrome (SS) is the leukemic variant of cutaneous T-cell lymphoma characterized by a triad of erythroderma, peripheral lymphadenopathy, and the presence of circulating atypical lymphoid cells (Sezary cells) in the blood. We present a 65-year-old female with a recent-onset schizophrenia, presenting with a 3-year history of recurrent generalized pruritus, erythroderma, and a 2-month history of recent-onset, mushroom-like skin tumors. Examination revealed generalized erythroderma affecting more than 80% of total body surface area and significant peripheral lymphadenopathy. Peripheral blood buffy coat examination showed Sezary cells which constituted more than 18% of total circulating lymphoid cells, and histology report of the wedge biopsy of skin tumor confirmed mycosis fungoides. The patient was commenced on cytotoxic chemotherapy with some improvement in symptoms. However, she relapsed 2 months later, with development of new fungating tumors and died from overwhelming sepsis. We report this case to highlight the occurrence of two comorbidities together, each of which may negatively worsen the progression of the other, and to report a case of SS as one of the rare causes of generalized exfoliative dermatitis. Thus, there is a need for early skin biopsy and histology in any elderly patient presenting with recurrent generalized pruritus and erythroderma.

Key concepts: Erythroderma, Generalized lymphadenopathy, Medicine, Mycosis fungoides, Inguinal lymphadenopathy, Skin biopsy, Dermatology, Pathology

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