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Case ReportsAbstract
Sweet’s syndrome (SS) is a neutrophilic dermatosis that can be classified into three forms based on etiology: classical (idiopathic), malignancy-associated, and drug-induced. However, another rare and severe variant, bullous hemorrhagic SS (BHSS), is distinguished by hemorrhagic bullae and dermo-epidermal separation. BHSS is associated with chronic myelogenous leukemia with elevated levels of pro-inflammatory cytokines such as IL-8, IL-17, TNF-α, and MMP-9. Herein is a case of a 70-year-old woman with multiple hemorrhagic plaques and bullae on the scalp, eyelids, fingertips, and tongue, raising suspicion for vasculitis or Wegener’s granulomatosis. Microscopic examination of the H&E slides demonstrated cryptococcoid neutrophils and histologic features including papillary dermal edema with dense neutrophilic infiltrate in the upper dermis seen in bullous hemorrhagic Sweet Syndrome. Direct Immunofluorescent studies were negative for IgG, IgA, C3, and Fibrinogen and “shaggy” for IgM basement membrane zone. This case emphasizes the importance of considering this rare variant and highlights the essential role of histopathology and immunofluorescence in distinguishing it from vasculitis and other blistering disorders to avoid potentially life-threatening complications.