Title : Is it really sweet? A challenging case of treatment resistant sweet’s syndrome in a patient with crohn’s disease
Abstract:
Sweet’s syndrome (SS), or acute febrile neutrophilic dermatosis, is a rare cutaneous manifestation in inflammatory bowel disease (IBD). It is characterised by a sudden onset of painful erythematous plaques, or nodules in the face, neck and upper extremities alongside pyrexia. This condition can improve with systemic corticosteroids and controlling IBD activity. However, treatment can be challenging when recurring SS flare-ups occur during well-controlled IBD. Herein, we present a 40-year-old female with stable pancolonic Crohn’s disease developing an acute onset of painful erythematous nodules on the face and forehead, with 38°C fever and leukocytosis. This occurred three weeks following administration of Upadacitinib (second-generation JAK kinase inhibitor). Conventional and biologic DMARDs were previously used but discontinued due to infective chest complications. A clinical diagnosis of neutrophilic dermatosis was confirmed with a skin biopsy result demonstrating dense neutrophilic dermal infiltrate. The symptoms improved within a day of high-dose oral Prednisolone 40 mg, once daily but it was difficult to wean off as it provoked SS flare-ups. Additionally, similar less severe skin lesion flare-up occurred in the past following Adalimumab (Humira) injections, suggesting the possibility of a dysregulated cytokine balance exacerbated by biologic medications. Subsequently, a trial of Anakinra was commenced which is currently helping taper the oral steroids without a flare-up so far. This case report highlights the complexity in treating multi-system pathologies and suggests SS flare-ups may not always directly correlate with IBD activity.
Audience Take Away Notes:
- New treatment option for treatment resistant Sweet’s syndrome which can cause a catastrophic impact unto patients’ lives if not controlled
- It can help health professionals broaden their knowledge on this condition enabling early and efficient management of this uncommon skin condition
- This case study can help provide a platform for new research opportunities to explore newer treatment strategies for treatment resistant Sweet’s syndrome
