Necrobiosis lipoidica arising in a port wine stain treated with topical ruxolitinib.

Abstract

We present a unique case of necrobiosis lipoidica (NL) arising within a port-wine stain (PWS), which, to our knowledge, has not been previously reported. NL is a rare granulomatous disorder often associated with diabetes mellitus, characterized by chronic inflammation and microvascular dysfunction. PWS, a congenital vascular malformation, results from defective endothelial differentiation and capillary malformation. The coexistence of these conditions suggests a potential link in their pathophysiological mechanisms, including inflammation, endothelial dysfunction, and shared signaling pathways such as mitogen-activated protein kinase (MAPK), phosphoinositide 3-kinase (PI3K), and tumor necrosis factor (TNF)-alpha. Our patient's NL lesions demonstrated improvement with topical ruxolitinib, a Janus kinase (JAK) inhibitor, after failing conventional therapies, including clobetasol, tacrolimus, and pulsed dye laser. To our knowledge, this is the first reported case of NL successfully treated with topical ruxolitinib monotherapy. This case highlights the emerging role of JAK inhibitors in managing granulomatous disorders and raises intriguing questions about the shared pathophysiologic mechanisms between inflammatory and vascular processes. Further investigation into cytokine dysregulation, immune responses, and targeted therapies for these overlapping pathologies could inform more effective treatment strategies and improve patient outcomes.