Dual diagnosis of histiocytoid Sweet s... - Sweet's Syndrome UK

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Dual diagnosis of histiocytoid Sweet syndrome and anti-neutrophil cytoplasmic antibody-associated vasculitis

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Shell567AdministratorSweet's Syndrome UK
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Dual diagnosis of histiocytoid Sweet syndrome and anti-neutrophil cytoplasmic antibody-associated vasculitis, APR 2022.

"Histiocytoid Sweet syndrome (H-SS) is a rare variant of acute febrile neutrophilic dermatosis. It is frequently associated with hematologic malignancies but has also been associated with certain inflammatory states. Here we report a case of concurrent anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and H-SS diagnoses."

"Our patient had a very rare combination of H-SS skin lesions in the setting of ANCA proteinase-3 granulomatosis with polyangiitis glomerulonephritis."

ADDITIONAL NOTE.

HISTIOCYTOID SWEET'S SYNDROME: In Sweet’s syndrome, mature white blood cells called neutrophils accumulate in the tissues. In histiocytoid Sweet’s syndrome, immature myeloperoxidase-positive cells and not mature neutrophils can be seen (mainly M2-like CD 163+macrophages). Myeloperoxidase is an enzyme that’s abundantly expressed in neutrophils.

The immature cells in histiocytoid Sweet’s syndrome can be mistaken for histiocytes; immune cells that destroy foreign substances and help fight infection. Sometimes, real or authentic histiocytes may also be present.

ANCA-ASSOCIATED VASCULITIS: Neutrophilic dermatoses, including Sweet's syndrome, have previously been recognized in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. ncbi.nlm.nih.gov/pmc/articl...

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Shell567 profile image
Shell567AdministratorSweet's Syndrome UK

What is ANCA? - Vasculitis UK. vasculitis.org.uk/about-vas...

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