SEZARY SYNDROME MIMICKING GENERALIZED PSORIASIS VULGARIS

Abstract

Background: Sezary syndrome is the one type of cutaneous T cell. This disease is characterized by reddish patches or plaques on the skin which extends to whole body into erythroderma, lymphadenopathy and presence of atypical lymphocytes called Sezary cells.Purpose: To know clinical manifestation, examination and management of Sezary syndrome which clinically resembles generalized psoriasis.Case: A man 60 years old came with scaly plaques reddish brown on almost of his body accompanied by lymphadenopathy on the supraclavicular lymph node right and left and accompanied by intense itchy also. Another clinical features were alopecia, palmoplantar hyperkeratosis, onychodysthropy, facies leonine without anesthesia on the lesion and without enlargement of peripheral nerve. From laboratory test, there is an increasing in the number of leukocytes, from the peripheral blood smear examination found Sezary cells and histopathology showed focal athrophy and acanthosis of the epidermis and dense infiltration of lymphocytes in the dermo-epidermal junction and superficial dermis. Case management: Patient received methotrexate (MTX) 3 x 5 mg (1 cylcle) with mometasone furoate 0,1% cream and CTM 3x1 tablet for adjunctive therapy. Methotrexte was discontinued because there are increasing of liver function and deterioration of patient’s condition. After 25 days of treatment, the patient got sepsis and then the patient died. Conclusion: Early onset of Sezary syndrome in this case is difficult to know because the clinical manifestation is similar with psoriasis vulgaris. Supporting examination such as laboratory test, blood smears and histopathology examination could help diagnosis. The presence of lymphadenopathy, atypical lymphocytes in the peripheral blood and extensive skin involvement reflecting the poor prognosis. The most common cause of death was sepsis.