Sweet Syndrome in Eosinophilic Granulo... - Sweet's Syndrome UK

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Sweet Syndrome in Eosinophilic Granulomatosis with Polyangiitis.

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Neutrophilic dermatoses, including Sweet's syndrome, have previously been recognized in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is an ANCA-associated vasculitis. Vasculitis is inflammation of the blood vessels. SEE COMMENTS FOR INFO ON THESE CONDITIONS.

This is a case of a 46-year-old man with asthma who developed a fever, shortness of breath, difficulty moving about, and a severe rash. Skin lesions were on his scalp. Blood tests showed a raised white cell count, including a high eosinophil count (absolute eosinophil count 7.75), high-titer myeloperoxidase antibody (MPO-ANCA), and positive perinuclear ANCA (p-ANCA). CT scan demonstrated large subpleural opacities (lung involvement). Nerve conduction studies were consistent with peripheral neuropathy.

The findings were consistent with EGPA, but the rash wasn't typical of this condition - often subcutaneous nodules with eosinophilic vasculitis. The biopsy showed dense neutrophilic infiltrate with dermal oedema indicating Sweet's syndrome (lots of white blood cells called neutrophils in the tissue; fluid leaking into dermis; normally an absence of vasculitis).

The patient’s symptoms improved with intravenous methylprednisolone, followed by oral prednisone and rituximab.

jrheum.org/content/47/7/1031

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Acute febrile neutrophilic dermatosis (Sweet's syndrome): dermnetnz.org/topics/acute-...

Cutaneous small vessel vasculitis: dermnetnz.org/topics/cutane...

Eosinophilic granulomatosis with polyangiitis: dermnetnz.org/topics/eosino...

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