Inflammation on Steroids: Uncovering Skin Clues in Adult Autoinflammatory Diseases

Ever heard of autoinflammatory disorders? They're like when your body's immune system goes a little haywire and overreacts to tiny, harmless signals. This isn't your typical allergy or infection. We're talking about a small army of neutrophils and macrophages causing too much inflammation. Unlike other immune disorders, these guys don't have anti-nuclear antibodies, which are like the body's little alarm bells for connective tissue diseases. Many of these disorders pop up due to inherited genetic mutations, especially in kids and young adults. But get this, there's a new kid on the block called VEXAS syndrome. This changes everything because it can happen later in life, showing that these mutations aren't always from birth. Some disorders, like adult-onset Still's disease and Schnitzler syndrome, are caused by a mix of factors and aren't just about one gene. You might be wondering, why should we care about these conditions? Well, many of them show up on the skin, either at the beginning or sometime during the disease. This is a big deal for doctors trying to figure out what's going on. Now, let's chat about a few examples. Adult-onset Still's disease (AOSD) can cause a nasty rash that comes and goes. Then there's VEXAS syndrome, which might bring on a painful skin ulcer. Schnitzler syndrome makes your skin red and itchy, as if it's under attack. Kikuchi-Fujimoto disease can make your lymph nodes swell up, and hemophagolymphohistiocytosis (HLH) can cause strange bruises all over your body.