What is Erythrodermic Psoriasis?
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Shiva: My name is Shiva Mozaffarian and I’m here today with Dr. Mio Nakamura from the University of Michigan, Department of Dermatology to discuss erythrodermic psoriasis. Dr. Nakamura is a board-certified dermatologist and Assistant Professor of Dermatology at the University of Michigan, School of Medicine, where she provides medical dermatology diagnosis and treatment for people of all ages and has a clinical interest in treating chronic inflammatory skin conditions such as psoriasis. Additionally, she maintains a strong interest in clinical research and serves as the Director of the Program for Clinical Research in Dermatology where she oversees patient recruitment and participation in clinical and translational studies to better understand various dermatologic conditions and treatments.
Welcome Dr. Nakamura. It’s such a pleasure having you on Psound Bytes today. We heard from Dr. Michael Lewitt in episode 142 about “types of psoriasis” that erythrodermic psoriasis occurs rarely, affecting 0.25 to 2 percent of those who have psoriasis. To help remind us about this type of psoriasis, can you please describe what erythrodermic psoriasis is? What are the most common symptoms?
Dr. Nakamura: Sure. First, thank you so much for having me today. I'm very excited and honored to be here. So erythrodermic psoriasis is an uncommon, serious and potentially life-threatening form of psoriasis where at least 75 to 90% of the skin is affected with psoriasis. Men are more likely to have erythrodermic psoriasis with a 3 to 1 male to female ratio and the average age of onset is about 54 years old. There are two types of erythrodermic psoriasis, the first is when a person with a history of psoriasis has worsening of psoriasis to the point that it's affecting almost the entire skin. This tends to be a slower process and responds better to treatment compared to the second type where someone has a rapid onset of psoriasis throughout the skin surface, which can be more serious and difficult to treat. This acute type requires urgent treatment. If the erythrodermic psoriasis is due to worsening or flare of preexisting plaque psoriasis, you'll see both the classic plaques of psoriasis, which are well demarcated, red scaly plaques, as well as newer areas of psoriasis which are red and thinner with very fine scale. In the second type, when the erythrodermic psoriasis comes up acutely, it happens quickly and looks like a thin, bright pink to red rash with very fine scale and in either case most of the skin surface is affected and the skin tends to be very warm. Some patients will have intense itching, some may have skin pain. You can also have both itching and pain. You can also have nail changes and diffuse hair loss can be seen too.
Shiva: So you mentioned that the acute form of erythrodermic psoriasis requires urgent treatment. Why is this the case? What happens in the body that raises the need for urgent care?
Dr. Nakamura: The skin is crucial for regulating body temperature and fluid levels in our bodies. When so much of the skin is affected as in erythrodermic psoriasis, it can affect your entire body. This is especially the case when it happens acutely, and your body doesn't have time to adapt. The symptoms can be anything from fevers, chills, sweats, and feeling tired, to having a fast heartbeat, lightheadedness and blood pressure abnormalities too. In very rare and severe cases, erythrodermic psoriasis can cause heart failure and even death. It's important to seek care urgently to avoid these complications.
Shiva: Definitely urgent. So why does erythrodermic psoriasis occur in the first place? What are the triggers or what predisposes someone towards developing erythrodermic psoriasis?
Dr. Nakamura: In more than a half of cases of erythrodermic psoriasis, there's a certain trigger, and there are many possible triggers for erythrodermic psoriasis. Triggers in our environment include sunburns, skin injury, alcohol use, and emotional stress. Physical stressors like illnesses and infections like HIV can also be a trigger. Medications like certain antibiotics can trigger erythrodermic psoriasis. If someone stops their psoriasis medications like methotrexate or steroids it can also result in erythrodermic psoriasis because the psoriasis rebounds and flares rapidly. We don't really know exactly why these triggers cause erythrodermic psoriasis, but they lead to abnormalities in the activity of immune cells in the skin. Some studies have shown that immune cell abnormalities in the skin that cause erythrodermic psoriasis are surprisingly different from those that cause other types of psoriasis. In chronic plaque psoriasis, the immune pathway called the TH1 and TH17 are involved. This pathway causes elevation and inflammatory proteins like TNF alpha and IL-17, but in erythrodermic psoriasis, a different pathway called the TH2 pathway, is more involved, which raises different inflammatory proteins like IL-4 and IL-10 in the skin. Interestingly, the TH2 pathway is known for being involved in eczema, which is a completely different skin disease. So erythrodermic psoriasis is likely a distinct subtype of psoriasis rather than just a really bad flare of plaque psoriasis.
Shiva: So say someone shows up in the ER or the urgent care center based on what you said previously. Is it possible erythrodermic psoriasis could be mistaken for other diseases? How is the diagnosis confirmed?
Dr. Nakamura: Yes, erythrodermic psoriasis can look like other skin conditions that cause erythroderma. Erythroderma is a term for a full body red rash. So other causes of erythroderma include atopic dermatitis or eczema and drug reactions. There are other rare conditions that cause erythroderma like pityriasis rubra pilaris and cutaneous T-cell lymphoma, among other conditions. There are certain clues to the patient’s history and the look of the rash that can help distinguish between these different skin conditions. But a skin biopsy can also be helpful to confirm the diagnosis of erythrodermic psoriasis and make sure it's not one of these other conditions.
Shiva: Good to know. So is the risk of infection greater in someone with erythrodermic psoriasis? And if so, what types of infection could occur?
Dr. Nakamura: Yeah, because the skin barrier is compromised in erythrodermic psoriasis, the risk of infection is greater. Superficial skin infections, like staph infections can happen, and a certain type of bacteria called streptococcus can cause a superficial skin infection called erysipelas. When the infection’s a bit deeper in the tissue, you can have cellulitis and as the infection goes into the blood stream, sepsis can occur, which is very serious and would require antibiotic treatment in an inpatient hospital setting.
Shiva: So given what you've just mentioned, it seems like erythrodermic psoriasis requires a rapid response. What treatments are initially given to stabilize symptoms? In the past, we've heard about the use of cyclosporine or infliximab. Has that changed?
Dr. Nakamura: Luckily, there are many treatment options for erythrodermic psoriasis today. As you've said, the key is for the treatment to work quickly to avoid complications. In traditional oral medications, like cyclosporine, can work very quickly and then it's still a really great option today. This medication is reserved for otherwise healthy people, especially those with good renal function (our kidney function). And this drug also requires frequent lab monitoring. Biologic medications like infliximab are good options too. There are other newer biologics aside from infliximab that also work well for erythrodermic psoriasis.
Shiva: You mentioned there are newer biologics that offer a more rapid response for treating erythrodermic psoriasis. Can you identify what those treatments are?
Dr. Nakamura: Right. Infliximab works quickly, but it's an IV infusion. Unlike the other biologics which are subcutaneous injections. So this can be a little bit inconvenient. Other biologics ah work well include ustekinumab, secukinumab, ixekizumab, and risankizumab. And these are known to work especially quickly, which makes them good options for erythrodermic psoriasis.
Shiva: So what other treatments are used to help bring erythrodermic psoriasis under control? Are topicals used at some point?
Dr. Nakamura: Yes, traditionally topical steroids have been used to treat erythrodermic psoriasis, especially if applied under occlusion, and this is called the modified Goeckerman therapy, and it can work very quickly and effectively. Topical steroids are also important for long term management of more stable and chronic types of erythrodermic psoriasis.
Shiva: And depending on the type, how long is the recovery period for erythrodermic psoriasis? What should someone expect once diagnosed?
Dr. Nakamura: With adequate treatment, the majority of patients with erythrodermic psoriasis will recover within a few weeks. Once the diagnosis of erythrodermic psoriasis is made, you may be admitted to the hospital or treated on an outpatient basis depending on the severity and whether or not you have any of the systemic symptoms that we discussed earlier. You should be started on a treatment right away and systemic symptoms should go away rather quickly once adequate treatment is started. Hopefully you'll start to see skin improvement in the first few days to a week. But it can take a few weeks until there are significant improvement in the skin. There can be relapse or recurrence of erythrodermic psoriasis, so having a long-term treatment regimen is important too.
Shiva: And what treatments should someone avoid if they have erythrodermic psoriasis?
Dr. Nakamura: Well phototherapy is a great treatment option for different types of psoriasis. It should be avoided in erythrodermic psoriasis because it can cause erythrodermic psoriasis to worsen. Some treatments for psoriasis, like methotrexate and acitretin, are really good long-term options, but they may not work quickly enough for erythrodermic psoriasis. And lastly, oral corticosteroids can give rapid relief over erythrodermic psoriasis but can result in a rebound phenomenon which means that the psoriasis might flare up when the medication is stopped even worse than before, so this is a controversial phenomenon though.
Shiva: And do you have any tips beyond what you just mentioned about treatments to help prevent erythrodermic psoriasis? Are there any early warning signs to watch for?
Dr. Nakamura: I would just emphasize that if you have a history of psoriasis and it's worsening rapidly due to a trigger or because your psoriasis medication was stopped, then I would make sure to seek care right away before it turns into erythrodermic psoriasis. Erythrodermic psoriasis is preventable and treatable.
Shiva: Certainly a good point to emphasize. Thank you, Dr. Nakamura for providing your insights and your expertise to the management of erythrodermic psoriasis. Do you have any final comments you'd like to share with our listeners today?
Dr. Nakamura: I think we've covered a lot. Thank you for the great questions and I hope the listeners found this informative.
Shiva: Thank you again for being here with us today and for providing such invaluable information about the recognition and management of erythrodermic psoriasis. I hope the information you provided today will reach those in need. For more information about types of psoriasis and treatment options contact our Patient Navigation Center by calling (800) 723-9166, option 1 or by emailing firstname.lastname@example.org . And finally, thank you to our sponsors who provided support on behalf of this Psound Bytes episode through unrestricted educational grants: AbbVie, Amgen, Bristol Myers Squibb, and Janssen.
We hope you enjoyed this episode of Psound Bytes™ for people with psoriasis and psoriatic arthritis. If you or someone you love has ever struggled with psoriatic disease, our hope is that through this series you’ll gain information to help you lead a healthier life and inspire you to look to the future. Please join us for another inspiring podcast. You can find this or all future episodes of Psound Bytes™ on Apple Podcasts, Spotify, iHeart Radio, Google Play, Gaana, and the National Psoriasis Foundation web page. To learn more about this topic or others please visit psoriasis.org or contact us with your questions or comments by email at email@example.com.
This transcript has been created by a computer and edited by an NPF Volunteer.
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