Abstract

Sweet Syndrome (SS) is an acute neutrophilic dermatosis distinguished by acute onset of tender plaques and nodules, classically associated with concomitant fever and leukocytosis. It is a non-vasculitic dermatosis and may, rarely, be accompanied by extracutaneous systemic manifestations. Neutrophilic dermatoses can be idiopathic or associated with underlying malignancies or manifestation of a drug reaction. Case Presentation: A 33-year-old male presented with leukocytosis and deep vein thrombosis accompanied with severe edema in his right upper extremity presented with persistent high fevers and acute onset of bullous skin erosions. His right upper extremity exhibited erythematous and edematous plaques with bullae, erosions and desquamation. Punch biopsy from the affected area revealed skin with mildly acanthotic epidermis with spongiosis and neutrophilic exocytosis. Papillary dermal edema along with a dense neutrophilic infiltrate in the dermis were also noted. Infectious workup including CMV and HSV I and II was negative. Due to findings on peripheral smear, a bone marrow study was performed that led to the diagnosis of atypical chronic myeloid leukemia. Usually, Sweet syndrome is seen as a result of chemotherapy in hematologic malignancies but this case highlights that neoplastic neutrophils can also manifest as cutaneous lesions.