title: Sweet syndrome-like neutrophilic infiltrate as initial presentation of acute myelogenous leukemia.
abstract: Sweet syndrome (SS) is a neutrophilic dermatosis that may be associated with malignancies, especially hematological. We describe the case of a 53-year-old woman with a clinical presentation suggestive of SS, accompanied by pancytopenia and a hypercellular marrow with signs of myelodysplasia. The histopathological findings were characterized as an SS-like cutaneous neutrophilic infiltrate with atypical myeloid cells, myeloperoxidase, and BCR-ABL+, which were absent in peripheral blood and bone marrow aspirate. The patient was treated with systemic corticosteroids with resolution of symptoms and relapse 3 months later when we tried drug withdrawal. Eight months later, the patient was admitted to hematology for a mature acute myelogenous leukemia with an FLT3 mutation. The patient successfully underwent medullar allotransplant and is now asymptomatic (5-month follow-up). This case describes a patient with an acute myelogenous leukemia presenting initially with heralding SS-like cutaneous neutrophilic infiltrate with atypical BCR-ABL+ myeloid cells, as a form of aleukemia cutis. Early recognition of this so-called aleukemic leukemia cutis may allow clinicians to intervene earlier, initiating effective treatment.
authors.affiliation: *Department of Dermatology, Hospital de Santa Maria (CHLN), Lisbon, Portugal; †Dermatopathologie Friedrichshafen, Friedrichshafen, Germany; and ‡Department of Hematology, Hospital de Santa Maria (CHLN), Lisbon, Portugal.
authors.forename: Ana Rita
authors.initials: AR
authors.lastname: Travassos
authors.forename: Luís
authors.initials: L
authors.lastname: Soares-de-Almeida
authors.forename: Heinz
authors.initials: H
authors.lastname: Kutzner
authors.forename: Lurdes
authors.initials: L
authors.lastname: Guerra
authors.forename: José
authors.initials: J
authors.lastname: Alves do Carmo
authors.forename: Paulo
authors.initials: P
authors.lastname: Filipe
authors.forename: Manuel Sacramento
authors.initials: MS
authors.lastname: Marques
mesh_terms: D005260:Female; D016044:Fusion Proteins, bcr-abl / Q000235:genetics; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000235:genetics / Q000473:pathology*; D008875:Middle Aged; D020556:Neutrophil Infiltration; D016463:Sweet Syndrome / Q000235:genetics / Q000473:pathology*
chemical_list: D016044:Fusion Proteins, bcr-abl