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Published on: November 21, 2018
An Uncommon Presentation of Sweet's Syndrome in Primary Myelofibrosis
Valentina Rago1,2, Valeria Brogna1,3, Fabrizio Cavalca2
1Department of Medicine and Surgery, University of Milano-Bicocca, Milano, Italy.
Abstract:
Sweet's syndrome (SS) is a rare neutrophilic dermatosis often associated with hematologic malignancies. Due to its infectious mimicry, diagnosis is frequently delayed. We report a case of primary myelofibrosis initially treated for suspected cellulitis, which progressed to necrotic lesions and refractory fever despite broad-spectrum antibiotics. A skin biopsy confirmed neutrophilic vasculitis consistent with SS. Prompt treatment with corticosteroids followed by ruxolitinib achieved rapid resolution by suppressing the underlying cytokine storm.
Insights
Sweet's syndrome, a rare skin condition, can mimic infections, delaying diagnosis. Early corticosteroid and ruxolitinib treatment effectively resolved symptoms in a primary myelofibrosis patient.
Area of Science:
- Dermatology
- Hematology
- Pathology
Background:
- Sweet's syndrome (SS) is a rare neutrophilic dermatosis.
- It is often associated with hematologic malignancies.
- Diagnostic delays are common due to its mimicry of infections.
Purpose of the Study:
- To report a case of Sweet's syndrome in a patient with primary myelofibrosis.
- To highlight diagnostic challenges and treatment outcomes.
Main Methods:
- Clinical case presentation.
- Review of patient history, symptoms, and treatment.
- Skin biopsy for histopathological confirmation of neutrophilic vasculitis.
Main Results:
- The patient presented with symptoms initially misdiagnosed as cellulitis.
- Necrotic lesions and persistent fever despite antibiotics indicated a more complex condition.
- Skin biopsy confirmed Sweet's syndrome.
- Treatment with corticosteroids and ruxolitinib led to rapid symptom resolution.
Conclusions:
- Sweet's syndrome can present atypically and be mistaken for infections.
- Early diagnosis and targeted therapy, including ruxolitinib for cytokine suppression, are crucial for managing SS associated with myelofibrosis.
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