S zary syndrome was first described in 1938, by S zary and Bouvrain. The classical
description of this syndrome consist of generalized pruritus, exfoliative dermtitis with
edema and thickening of the skin, ectropion, leonine facies, alopecia, dystrophic nails,
keratoderma of the palms and soles, and lymphadenofathy associated with leukocytosis
and abnormal mononuclear cells.
We present a case of S zary syndrome, in a 50-year-old male farmer with generalized
exfoliative erythroderma, keratoderma of palms and soles, generalized lymphadenopathy,
hepatomegaly, and pruritus, as clinical features, and severe leukocytosis (W.B.C. : 66,000/
㎣ of blood) with increased atypical mononuclear cells(21,120/㎣ of blood) in the
peripheral blood, of which most were identified as cerebriform mononuclear cells with
electromicroscopy as hematologic features. Histopathologic findings of the skin lesions
revealed hyperkeratosis with parakeratosis and slight acanthosis of epidermis, moderate
to marked mononuclear cells mixed with a few atypical cells in the papillary and upper
dermis with epidermotropism. Axillary lymph node biopsy findings showed loss of
normal architecture and marked infiltration of mononuclear cells with many atypical
cells.
Chemotherapy with vincristine, cyclophosphamide, adriamycin, bleomycin, and
prednisolone, was done. Two weeks later, the exfoliative errthrodermic lesion and
pruritus were nearly subsided, and a few atypical lymphocytes(504/㎣) were observed in
peripheral blood smear.
|