Case study reports acute febrile neutrophilic dermatosis, highlighting genetic factors and cytokine levels.
Background. Sweet syndrome is a rare inflammatory disease from the group of neutrophilic dermatoses, characterized by proinflammatory cytokines hyperproduction. Case description . A child, 1 year 3 months old, with referral diagnosis of “Pyoderma gangrenosum” was diagnosed: «Acute febrile neutrophilic dermatosis. Sweet syndrome». Molecular genetic testing was carried out to clarify the diagnosis: whole-exome sequencing of venous blood from the patient and his mother. As a result both of them had nucleotide variant c.3061C>T in exon 26 of the UBA1 gene. Step-up therapy with methylprednisolone, dapsone, tofacitinib, anakinra, and ruxolitinib was administered due to pathological skin process severity, high interleukins 1 and 6 levels, and according to the experience in management of such patients described in medical literature. Sustained remission of the skin pathological process was noted on treatment. Conclusion . Case study of acute febrile neutrophilic dermatosis (Sweet syndrome) is presented. Results of successful drug therapy are shown.
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Murashkin et al. (2025) studied this question.
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