My Battle With Behcets

I am a zebra. This is a term for people with rare diseases. When you hear hoof beats think horse, not a zebra. This is what doctors say to imply that patients likely have something common. But sometimes hoof beats are zebras especially if you live near the zoo. Sometimes patients have the rare disease. I was diagnosed with Behcet’s disease after months of symptoms that didn’t make sense and didn’t initially lead to any diagnosis. I figured out my diagnosis through countless hours of meticulous research (and months of misery and continued dismissal). I brought my research and images of my rashes and ulcers to a rheumatologist, after months of waiting, and he said “you absolutely have Behcet’s disease”. This was the first time I actually felt validated since my symptoms started. I suddenly had an answer.

What is Behcet’s disease? It is an autoinflammatory syndrome with components of vasculitis and spondyloarthopathy (type of arthritis). It is chronic and has onset most commonly in young adulthood. There is no blood test that can diagnose this disease, although many people will have elevated inflammatory markers (CRP, ESR) and a large proportion have a genetic component by having HLA B51. Most people get diagnosed when other more common autoimmune diseases (like Lupus, rheumatoid arthritis, other types of vasculitis) are ruled out; these other diseases usually have antibodies identifiable in blood. This means Behcet’s disease is a clinical diagnosis, where the patient has to meet diagnostic criteria to be diagnosed. There is a set criteria for diagnosis, but there are many other manifestations of the disease that are not described. Behcet’s disease is very heterogenious in this way.

What is the diagnostic criteria? There are actually a few different criterion, and the criteria that is used depends on preference. They are all pretty similar, some more strict than others on the presence of certain symptoms. The one most commonly used in the US is the international criteria for behcet’s disease. I’ll link it below.

The ICBD says:

My initial symptoms were nonspecific. I had joint pain very first, but my doctor couldn’t figure out the cause. I would have tendinitis all over, making it hard to weight lift, which I loved to do. Then I started having weird rashes, one type where I would flush for hours all over my body and then it would just disappear. I would have folliculitis appearing as small bumps at various hair follicles. Initially this was mild and I did not think much of it. My facial acne got extremely bad after years of clear skin after puberty acne. I attributed to moving back to high elevation with very dry air after living 4 years in Portland Oregon where it would rain 9 months out of the year. Then I had surgery on my ankle. My symptoms exploded. I developed folliculitis that would ulcerate. The ulcers took weeks to begin to heal and left behind hyperpigmented scars. My dermatologist had no idea what the cause was, and he tried multiple antibiotics prior to doing a biopsy. The biopsy came back as pretty nonspecific inflammation around the hair follicle and nearby blood vessels. I also had livedo reticularis on my legs, making them appear lacy and purple. My feet were constantly cold. I would have tingles dancing across my skin. Eventually I developed the same bumps and ulcers on my chest, arms and back. I had giant ones on my mons pubis. Then I had my first genital ulcer. My dermatologist put me on steroids, which finally helped. My ankle incision had stopped healing when this rash started, but it finally healed the rest after starting steroids. My skin stopped ulcerating and the old ulcers cleared. I stopped having flushing. I felt better. But as I tapered off the steroids, my symptoms returned with a vengeance. I did a second steroid taper and started methotrexate, treating what dermatology labeled leukocytoclastic vasculitis: a skin limited vasculitis that is most often self-limited. It was supposed to go away on its own. It did not.

At the end of my summer break before my second year of medical school, I ended up being admitted to the hospital. Over the course of a week at the end of my second steroid taper, my rash had gotten worse, I had horrible fatigue, my joints hurt, I had a headache and numbness on my face, nerve pain in my legs, and I had acute weakness in my right hand and legs. I also was at my lowest weight, having lost 25 pounds in 6 weeks unintentionally. I went to the ER and they admitted me for further workup. For 3 days they did imaging and so much blood work and an EMG, none of which was helpful in eliciting a diagnosis. I was told the neurologic symptoms were conversion disorder, aka functional neurologic disorder. Because they couldn’t figure out what was wrong, I was labeled with a neuropsychiatric disorder. I was told the ulcers were from picking my skin. The other rashes were dismissed, but not for any valid reason. Ultimately, I was dismissed and invalidated. I was discharged feeling defeated. They had stopped the methotrexate and steroids in the hospital, so I was anxious. A few weeks later, my flare cleared up. Most of my skin had cleared and the neurologic symptoms dissipated. I was truly relieved. But it was short lived. About 6 weeks later, those symptoms and new ones appeared.

It was December, and I got super sick with bronchitis and a sinus infection. As I recovered from my illness, my joints started hurting and my wrist swelled. I had tendon pain in my knees. My eyes were red and irritated. I was exhausted. I had pustules form in different places over my body. The folliculitis and livedo reticularis returned. I had ulcers around and on my genitals. I would get full fast and start feeling my food come up my throat, symptoms that would later be attributed to gastroparesis. My headache returned with numbness and tingling in various locations. I developed an ulcer where I had an IV poke, consistent with a pathergy response. And then I developed a massive ulcer next to my tonsil. I noticed several smaller ulcers scattered in my mouth. I did not understand why I had so many ulcers. I thought about it and realized this was not my first ulcer outbreak. I had a few oral ulcers during the months prior, but really before 2022 I never had canker sores (aphthous ulcers). I had ulcers during the same time as my joint pain and various other nonspecific symptoms when I thought back in time. Then I started googling about recurrent aphthous ulcers, trying to see if any cause fit my situation. Then I came across Behcet’s disease, a disease that I had never heard of before. Other than uveitis, the criteria fit perfectly. I brought my findings to my upcoming appointment with a rheumatologist. I was so fortunate to be able to see the same doctor that took care of my mom who has rheumatoid arthritis. He took one look at my pictures and my skin and nodded in agreement, “you definitely have Behcet’s!” Little did I know, having a name to a condition was only the tip of the iceberg. There was so much more to come, but I’ll save that for another post. Otherwise this blog post will become a book!