Rare Case of Neutrophilic Dermatosis in Lung Transplant Recipient

Abstract

Introduction Sweet Syndrome is a rare neutrophilic dermatosis with systemic symptoms typically including malaise, fevers, and a characteristic cutaneous eruption. To our knowledge, it has not been described post lung transplantation. Case Report A 65 year old man with idiopathic pulmonary fibrosis received a bilateral lung transplant with basiliximab induction. His immunosuppression included mycophenolate mofetil, tacrolimus, and prednisone 20 mg daily. Nine weeks post transplant, he developed cyclical fevers to 38.4°C, malaise, myalgias, and neutropenia. Outpatient testing noted Stenotrophomonas maltophilia in bronchoalveolar lavage and a left leg deep vein thrombosis. He was treated with levofloxacin and tinzaparin. Despite therapy, fevers continued, and the patient was hospitalized. Granulocyte colony stimulating factor (G-CSF) 480 mcg was administered on admission and on post-admission day four. Hours after his first dose, he developed an abrupt onset of annular, red-violaceous, edematous plaques on his anterior neck, chest, and axillae with oropharyngeal mucosal ulceration. Infectious workup including blood culture, bone scan, WBC scan, and TEE returned negative. Tissue culture for fungi, mycobacteria, nocardia, and viral pcr from oral lesions were subsequently negative. Autoimmune disease was ruled out with a comprehensive immunologic evaluation. Skin biopsy showed immature histiocytoid myeloid cells and neutrophils consistent with histiocytoid Sweet Syndrome. Colchicine 0.6mg BID was initiated for treatment. Post-transplant lymphoproliferative disorder as a cause of Sweet Syndrome was ruled out with PET CT. Further doses of G-CSF were avoided. Slow resolution of constitutional symptoms and mucocutaneous findings occurred over several weeks. The patient developed GI intolerance to colchicine and was transitioned to prednisone 30 mg daily and colchicine 0.6 mg daily for ongoing management. Summary The patient s fevers and constitutional symptoms likely represented prodromal Sweet Syndrome, with exacerbation after administration of G-CSF. Sweet Syndrome has been documented after bone marrow transplant, but only one case, in a renal recipient, has been published following solid organ transplantation. Treatment options include high-dose prednisone, colchicine, dapsone, and potassium iodide. Refractory cases have been treated with IVIg or anakinra.