engleski

Sweet´s syndrome – a window to underlying malignancy

Introduction: Sweet's syndrome is commonly characterized by fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis. The malignancy-associated Sweet's syndrome can occur as a paraneoplastic syndrome in patients with an established cancer or individuals whose Sweet's syndrome-related hematologic dyscrasia or solid tumor was previously undiscovered ; it is most commonly related to acute myelogenous leukemia. Case report: A 61-year-old female patient with medical history of type II diabetes on insulin therapy presented with sudden onset of erythematous, annular, partially infiltrated skin lesions on chest with spreding on the back and extremities. One week before the onset of skin changes, patient had fever up to 38.5 °C, weakness, and weight loss. Laboratory findings included leukocytosis with neutrophilia (L 9.8, neu 75.3%), elevated sedimentation rate and CRP. Clinical presentation and laboratory findings were in accordance with the diagnosis of Sweet`s syndrome (acute febrile neutrophilic dermatosis). She had been treated with systemic corticosteroid therapy with prompt improvement after the initiation of therapy. Skin biopsy finding was also in accordance with the diagnosis of Sweet`s syndrome (orthokeratotic and focally parakeratotic, acanthotic epidemis with mildly irregularly prolonged epidermal papillae and infiltrates of neutrophils and mononuclears in the dermis.). Direct immunoflorescence did not detect imunoglobulines nor complement deposites. After finishing dermatologic treatment, the patient was referred to hematologist who extended the diagnostic work-up - JAK 2 mutation was negative and bcr - abl mutation positive, which is highly indicative for chronic myeloid leukemia. The patient is currently awaiting bone marrow biopsy to confirm the hematologic disease. Conclusion: This case is a good example how dermatologic conditions can facilitate the diagnosis of underlying malignant disease and thus enable its timely treatment.

sweet syndrome ; neutrophilia ; erythematous lesions ; paraneoplastic syndrome

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