A Case of Bullous Sweet's Syndrome Associated With Esophageal Adenocarcinoma

Abstract

Sweet's syndrome (SS), or acute febrile neutrophilic dermatosis, characteristically presents with fever, dermal neutrophilic infiltrates, and neutrophilia. It typically manifests as tender erythematous plaques; however, various variants are documented, including bullous. Malignancy-associated Sweet's syndrome (MASS) can present as a paraneoplastic syndrome in those with established cancers or with undiagnosed malignancies. We present a 72-year-old male with a three-day history of a progressive bullous, erythematous papular rash starting on his right forearm and spreading to his extremities, trunk, palms, and soles. It was mildly pruritic but nontender. He had no recent febrile illnesses. On examination, the rash was violaceous with tense bullae overlying edematous targetoid papules coalescing into plaques. Histopathologic analysis of punch biopsies from his abdomen and thigh demonstrated dense inflammatory infiltrates of neutrophils, eosinophils, histiocytes, and lymphocytes, suggestive of neutrophilic dermatosis, or Sweet's syndrome. He was treated with prednisone 1 mg/kg with improvement in his cutaneous symptoms, and a malignancy workup was initiated. Blood work showed elevated free kappa, lambda light chains, lactate dehydrogenase (LDH), and C-reactive protein (CRP) levels. A positron emission tomography (PET) scan revealed lesions in the esophagus and kidney. He was referred to Heme/Onc, GI, and Urology. He was diagnosed with esophageal adenocarcinoma stage IIb and a renal mass. He has since completed neoadjuvant chemotherapy and radiation, is s/p robotic Ivor-Lewis esophagectomy with no evidence of residual carcinoma on pathology, and is undergoing surveillance with imaging every three months for his renal mass. This case highlights the importance of rapid identification of MASS and the impact dermatologists can make in getting these patients the potentially lifesaving care they need.

Keywords: bullous; esophageal adenocarcinoma; malignancy; neutrophilic dermatosis; sweet’s syndrome.