Histiocytoid Sweet's Syndrome: A localized cutaneous proliferation of macrophages frequently associated with chronic myeloproliferative disease.

BACKGROUND: Histiocytoid Sweet's syndrome was originally described as cutaneous lesions of Sweet's syndrome where the infiltrate is mostly composed of histiocytoid mononuclear cells. The putative cell has been interpreted as an immature neutrophil based on the intense expression of myeloperoxidase.

METHODS: To better understand the nature of the infiltrate and potential mechanisms leading to this distinct form of cutaneous inflammatory cell influx, thirteen cases of histiocytoid Sweet's syndrome, encountered in the routine and consult practice of one of the authors, were studied. The clinical features and microscopic findings are summarized.

RESULTS: The study comprised eight men and five women aged from 23 to 80. There was a significant association with underlying myeloproliferative disease. In particular, five patients had underlying myelodysplastic syndrome. One patient had unspecified chronic myeloproliferative disorder and another had AML. Two cases were triggered by drug therapy (Cox-2 inhibitors). One patient had familial Mediterranean fever. The eruption was asymptomatic and an aggressive clinical course was not observed in most cases. Skin biopsies were composed of striking angiocentric and intersititial mononuclear cell infiltrates, often accentuated in the deeper dermis and subcutaneous fat. There was marked leukocytoclasia. Neutrophils were sparce or absent. These cells were strongly positive for CD163 and either expressed CD16 or myeloperoxidase. Variable positivity for myeloid dendritic cell markers including CD11c, BDCA-3, TCL1 oncogene, MXA and CD123 was observed.

CONCLUSION: The histiocytoid cells of histiocytoid Sweet's syndrome define a novel subset of activated monocytes. This variant of Sweet's syndrome has a significant association with underlying myeloproliferative disease.