Lenalidomide as a Steroid Sparing Agent in a Myelodysplastic Syndrome Patient with Refractory Sweet Syndrome

Abstract

Malignancy associated Sweet syndrome (acute febrile neutrophilic dermatosis) accounts for approximately 20% of all cases of Sweet syndrome and is characterized by painful, erythematous inflammatory papules and nodules that can precede, coincide or follow malignancies with predilection for hematologic malignancies (mainly acute myeloid leukemia and myelodysplastic syndrome). Corticosteroids are the cornerstone of treatment with exquisite response which has made many clinicians to include steroid responsiveness as one of the diagnostic criteria. However, recurrence can occur with tapering of steroids often needing a suppressive prophylaxis with unacceptably high dose of steroid for longer term. In addition, recurrence following tapering of steroids is a unique feature of malignancy associated Sweet syndrome occurring in two-third of patients. There is a paucity of effective steroid sparing strategy in the treatment of this disorder. Here we report a patient with myelodysplasia and refractory sweet syndrome successfully treated with low dose lenalidomide with resolution of skin lesions, improvement in hemoglobin level, allowing rapid and successful tapering of steroid and improvement in hyperglycemia. Immunomodulatory agents such as lenalidomide may have activity against sweet syndrome associated with myelodysplastic syndrome and therefore deserve further clinical investigation.