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Histiocytoid Sweet Syndrome
A Dermal Infiltration of Immature Neutrophilic Granulocytes
Luis Requena, MD;
Heinz Kutzner, MD;
Gabriele Palmedo, PhD;
Marta Pascual, MD;
Jesús Fernández-Herrera, MD;
Javier Fraga, MD;
Amaro García-Díez, MD;
Evaristo Sánchez Yus, MD
Arch Dermatol. 2005;141:834-842.
Objective To describe a series of 41 patients with fresh lesions of Sweet syndrome in which the histopathologic study demonstrated an inflammatory infiltrate mostly composed of histiocytoid mononuclear cells.
Design Histopathologic, immunohistochemical, and cytogenetic studies of the inflammatory infiltrate in a case series of histiocytoid Sweet syndrome.
Setting University departments of dermatology and a private laboratory of dermatopathology.
Methods Conventional histopathologic study as well as immunohistochemical investigations were performed using the alkaline phosphatase antialkaline phosphatase technique with a large panel of antibodies. In some cases, fluorescent in situ hybridization studies were performed to investigate the presence of the bcr/abl gene fusion.
Results Immunohistochemical studies demonstrated that most cells of the infiltrate showed immunoreactivity for CD15, CD43, CD45, CD68, MAC-386, HAM56, and lysozyme, which is consistent with a monocytic-histiocytic immunoprofile. However, intense myeloperoxidase reactivity was detected in most of the cells with histiocytic appearance, which raised the possibility of specific cutaneous involvement by myelogenous leukemia. Nevertheless, cytologic peripheral blood examinations, fluorescent in situ hybridization studies to investigate the bcr/abl gene fusion, and follow-up of the patients, taken all together, ruled out this possibility.
Conclusions This case series demonstrates that some fresh cutaneous lesions of Sweet syndrome are histopathologically characterized by an infiltrate mostly composed of cells that may be misinterpreted as histiocytes, when in fact they are immature myeloid cells. We named this histopathologic variant histiocytoid Sweet syndrome, which should not be mistaken with leukemia cutis or other inflammatory dermatoses that are histopathologically characterized by histiocytes interstitially arranged between collagen bundles of the dermis.
Author Affiliations: Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma (Dr Requena), and Departments of Dermatology (Drs Pascual, Fernández-Herrera, and García-Díez) and Pathology (Dr Fraga), Hospital Universitario La Princesa, Universidad Autónoma, Madrid, Spain; Dermatohistopathologische Gemeinschaftslabor, Friedrichshafen, Germany (Drs Kutzner and Palmedo); and Department of Dermatology, Hospital Clínico San Carlos, Universidad Complutense, Madrid (Dr Sánchez Yus).
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