An Evidence-Based Review of Perniosis (Chilblains): Therapeutic Strategies and Integration With Raynaud's Syndrome...

Perniosis (chilblains) is an uncommon inflammatory condition characterized by erythematous to violaceous papules or plaques typically affecting acral surfaces. The pathogenesis remains poorly understood, and its management remains challenging due to the limited evidence available. This comprehensive review examines evidence-based therapies for primary and secondary perniosis, informed by observational studies, case series, and trials on similar conditions such as Raynaud's phenomenon. We synthesize treatment options for managing inflammation, alleviating symptoms, and preventing recurrence, including topical and systemic vasodilators, corticosteroids, selective serotonin reuptake inhibitors (SSRIs), antimalarials, and antithrombotic therapies. A focus is placed on systemic vasodilators, including nifedipine, and topical therapies like nitroglycerin, which have shown efficacy in improving peripheral perfusion. Additionally, corticosteroids are used to address inflammation, although their effectiveness is debated in randomized controlled trials. Antimalarials and SSRIs, notably fluoxetine, also show promise, particularly for secondary perniosis. Despite the absence of high-quality randomized controlled trials, therapeutic recommendations have emerged from case reports and studies on related vascular conditions. The role of botulinum toxin, though under investigation, remains inconclusive for primary perniosis, but may be considered in cases of secondary perniosis or coexisting vascular issues. This review underscores the need for individualized treatment approaches and highlights the importance of future research to refine management strategies and enhance patient outcomes.