The Mystery of Granuloma Annulare

By Braiden Rex-Johnson

Television advertisements for well-known disorders such as psoriasis, rheumatoid arthritis, and asthma tout new wonder drugs that can clear plaque-ridden skin, improve the pain of damaged joints, and help people breathe more easily, respectively. But for sufferers of granuloma annulare (GA), there is no magic panacea that will stop the unsightly red rings, violet patches, oozing plaques, and painful nodules that can pop up anywhere on the body.

My own case of GA began in the spring of 2017. I was 61-years-old when a red ring, about the size of a quarter, popped up on the back of my right hand. The ring was slightly raised and a bit itchy. At first, I dismissed it as an insect bite or a pesky skin eruption and hoped it would just go away. But on an otherwise ordinary Saturday afternoon, while we were sharing fajitas at our favorite Mexican restaurant, my husband asked about the strange circle on my hand. It was then that I realized I needed to see a doctor STAT.

My primary-care physician thought the circle might be ringworm. But when a course of antifungal cream didn’t help, she prescribed a strong steroid cream. Even that didn’t faze it. And within weeks, I watched helplessly as red spots about the size of nickels started appearing on the backs of my pale and—until then—blemish-free legs. I watched in growing alarm as a smaller red spot sprang up on the outside of my left knee; then one on my right elbow. My skin was turning into a Modernist painting before my eyes as the angry red bumps spread over my extremities.

What were these strange spots? What if they spread to my face? I had always taken good care of my skin, choosing unscented products, moisturizing my body, and slathering on sun screen, so why was it turning against me now?

I made an emergency appointment with my long-time dermatologist. He agreed that my condition was most likely GA. To confirm the diagnosis, he anesthetized one of the most prominent bumps, then “punched” a small plug from its center. After examination by a pathologist, this “punch biopsy” confirmed that I had GA, and all of this just six weeks after the pesky red ring first appeared.

My dermatologist suggested starting intralesional corticosteroid injections with a drug called Kenalog. He said he hoped the under-the-skin injections would make the bumps go away, or at least calm the inflammation and help them fade over time.

Injections?!?! I had always been afraid of needles, and there were so many places to inject. The thin needles in the Kenalog-filled syringes moved over my body like a swarm of angry bees stinging their way across my torso. Sensitive places like the backs of my knees, under my breasts, and near my groin especially hurt. Sometimes I flinched and grimaced, unable to control my body movements.

To help ward off the pain, I often held my breath and pretended I was elsewhere. When the torture was over, I looked down at the examination table and saw red spots—my blood—spattered on the protective white paper where I had just laid. Although painful and unpleasant, the “jabs” became a necessary evil. I remained ever hopeful that they would make my GA go away, since my dermatologist kept predicting that it would most likely "burn out" within a year or two.

But it didn’t. The injections helped flatten the bumps and lighten the patches, but new lesions would simply pop up in different places. One time, after undergoing what seemed like hundreds of “jabs,” my doctor said that injecting me “was like a game of whack-a-mole.”

Granuloma annulare is an ugly disease, both literally and figuratively, affecting both mind and body. The uncertainty and unpredictability of this disorder—knowing it is roiling deep inside your body, ready to spring up anywhere and at any time—is one of the most unsettling and frustrating things about it.

Somehow, my precious husband of 40 years manages to see past my imperfect exterior. And a kindly nurse once said one of the patches looked like a butterfly tattoo! Still, I can’t imagine being diagnosed as a child, trying to navigate the dating scene, or going through GA alone. I am thankful we live in the Pacific Northwest, where I can hide my spots most of the time.

Since my diagnosis more than four-and-a-half years ago, I have learned a lot about GA, which translates from Latin as “inflamed ring” and is more correctly known as necrobiotic papulosis. The “inflamed rings” of GA were first reported in 1895 in the British Journal of Dermatology. According to a recent article in the American Journal of Clinical Dermatology, it is a rare disease, affecting just .04% of the United States population. Women are three times more likely to suffer from it than men, it most often occurs in the fifth decade of life, and is more prevalent among Caucasians.

The exact cause of GA is unknown, although some believe it is a result of trauma or injury to the skin (such as an insect bite), sun exposure, viral infections, or vaccinations. People with GA sometimes also suffer from thyroid disease, diabetes mellitus, and autoimmune diseases such as rheumatoid arthritis and lupus. The good news is that GA is neither cancerous nor contagious. The bad news is that some people experience pain, itching, burning, and perforations in their skin.

Granuloma annulare most often appears on the hands, wrists, feet, and ankles, although sometimes even on the face. It comes in different forms. The localized type often occurs in children, the rings are confined to one area, and sometimes it goes away spontaneously. Subcutaneous GA, also mainly seen in children, is less common and appears as rubbery lumps.

Perforating GA forms crusty yellowish plaques, mostly on the hands. The patch type presents as pink, red, or purplish patches and usually affects females over the age of 50. Generalized, or disseminated GA is diagnosed when a patient has more than 10 spots. More common in adults, generalized GA tends to be more persistent, is difficult to treat, and can last for decades, sometimes never resolving.

Because the underlying cause of this disorder is unknown, finding a gold-standard treatment that works for every patient is difficult. I have tried first-line options, including steroid creams and injections, and a third-line treatment, phototherapy (treatment with strong lights) without success. Through my research, I learned that certain prescription drugs have helped resolve some recalcitrant cases of GA. However, many dermatologists (including my own) are reluctant to prescribe them because they have not received FDA approval for use in GA, clinical trials are small or nonexistent, and side effects can be serious.

But after suffering for so long, I was getting desperate. And when my husband said he would be willing to “go to Mayo Clinic or anywhere else in the world to get me fixed,” I knew I had to do something drastic. Thankfully, we didn’t have to travel far afield. In January 2022, I found a dermatologist at a local teaching hospital who was willing to prescribe an oral drug called Hydroxychloroquine (yes, that Hydroxychloroquine!). Although it is more commonly used to prevent or treat malaria, rheumatoid arthritis, and lupus, Hydroxychloroquine has shown promise in patients with tough cases of GA.

My new doctor explained that while steroid injections can help, they don't address the underlying cause of GA. After explaining possible side effects (atrial fibrillation and retinal problems) she advised me to start taking Hydroxychloroquine twice a day for four to six months, and return for a three-month check-up.

I was overjoyed! If it worked, it meant no more fiddling around with creams, injections, or light therapy. But the first doses were tough. Nausea, dizziness, and unsettling dreams lasted close to a week, but eventually my body adapted. I am already seeing improvement, but even if Hydroxychloroquine doesn’t work or eventually stops working, my doctor said there are other drugs to try, always walking the fine line between side effects and efficacy.

Late last year, I discovered the Granuloma Annulare Support Group, a private Facebook group that was started in 2015 and now numbers close to 8,000 members from across the globe. Although this is a group none of us ever wanted to join, members are compassionate and caring. Sharing stories and photos has improved my mental attitude and helped me realize I am not alone. Misery loves company. When members were asked to describe GA in one word, the answers were heartbreaking: Evil, annoying, constant, perplexing, creeping, depressing, life-changing, relentless, unwanted.

Granuloma annulare is all of these and more. Yet the promise of systemic drugs offers GA sufferers something that is often in short supply: hope. And maybe someday, GA sufferers like me will be able to turn on the television and exult in advertisements that tout our very own wonder drug.

 

Braiden Rex-Johnson is a retired food-and-wine writer, the author of seven published cookbooks, and the owner/founder of www.SeattleWaterfrontWebcam.com.

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Braiden Rex-Johnson