Patient Journey to Diagnosis of PN

Raj Chovatiya, MD, PhD: Both of you run very busy clinics, where people are referred for a lot of complicated cases. Dr Elmariah, tell me what brings someone to see a specialist, or a dermatologist in general, who maybe has PN [prurigo nodularis] or something in that realm. How would you rate the awareness that’s out there in terms of dermatologists and patients?

Sarina B. Elmariah, MD, PhD: Those are all great questions. The thing that brings most patients in the door is usually frustration. Not only with their disease, but also often with the medical system and some of their previous physicians, whether it be their primary care doctors, neurologists, their family doctors, or certainly their dermatologists when they’re told to just stop scratching. Because that does not acknowledge only the burden of the disease but also the pathophysiology of the disease. Many patients come with this frustration of not having their symptoms controlled, and they feel misunderstood and like the burden of their disease is underappreciated.

Whenever a patient comes to me, the first thing we talk about is just a definition, a diagnosis. Half the time, they’re not given one, as we said. Patients can be told they have many different things or nothing at all. We try to lay that foundation first: This is a disorder, and there are other people out there. I’ll say to them, “I understand you haven’t been successfully managed, partly because until recently, we haven’t understood the disease. But here’s the approach we’re going to take.” That ends up building such trust and appreciation from the patient, even if they still are frustrated by the next few weeks, as we’re trying to get whatever their most burdensome symptoms are under control—sometimes months, even. I know we’ll talk about that in a bit, but patients come with many expectations and many kinds of emotional hurdles we need to tackle.

Raj Chovatiya, MD, PhD: What are some of those steps in your opinion, Dr Kwatra? Say you have somebody come into their primary care physician and they end up with you. What’s happening in between, before they may end up seeing you?

Shawn Kwatra, MD: I totally agree with Dr Elmariah’s comments. Getting the right diagnosis is a huge hurdle. They’ll see a primary care provider; they’ll get a random diagnosis—like rash and not otherwise specified—and they’ll potentially get some topical steroids. And then often, they’ll just go many years in between being completely mismanaged. Sometimes they’ll see dermatologist practices with providers who may not be super familiar with the disease. And there’s a variety of perspectives on the disease, because many years ago, when many of our dermatologists trained, the thought was that PN was all in your head. I’ve had patients referred to me who said they’ve seen dermatology-based providers, and they’ve been told not to scratch themselves. They’ve been told to see a psychologist, and they’ve gone years with the intense itch and sleep disruption.

Often, there are tiers before we even do anything, just to label what patients have and to say, “This is your diagnosis.” I can say that happens fairly regularly. I think that highlights how we have a very big problem with disease awareness and disease recognition, even among dermatologists. And it’s not their fault, because when they were training, they were told that this is all in the head. So it’s rapidly progressing our knowledge here. And I think, even from the primary care providers to some of the dermatologists, we still have to fill in the gaps for those providers.

Sarina B. Elmariah, MD, PhD: I just wanted to make 1 additional point. These patients have sought care with so many different providers, as Dr Kwatra mentioned, and sometimes multiple dermatologists before the diagnosis is explained and discussed with them, and they have had the battery of workup for itching. I want to be clear that PN is a very distinct entity in and of itself. Chronic itch is frustrating for many patients, but there is a distinct entity of PN. And often, these patients will still have the full battery of workup. Often, they’ll have tried multiple medications, multiple sedating antihistamines and immunosuppressive drugs, and some will be put on other psychiatric medications that may or may not help them, so there’s that built-in frustration. Because even though we define anything chronic as dealing with for over 6 weeks, most of these patients have suffered for years, so they come with a lot of clinical baggage.

Raj Chovatiya, MD, PhD: As Dr Kwatra put it, you can’t really blame providers who maybe weren’t necessarily trained in what we understand of the disease, but there are other things that could be on the differential here, too. It’s not necessarily the most straightforward diagnosis in the world, because as you mentioned, there are a bunch of reasons why someone can have chronic pruritus, and those need to be thoroughly explored. Even morphologically, you can see diseases ranging anywhere from something in the lichenoid family to something more like pemphigoid to something that is truly atopic dermatitis, and everything in between that can potentially be confused with that diagnosis of PN.

This is going to hearken back to some of that heterogeneity Dr Kwatra started talking about. Despite the name, it’s really not some monolithic disorder, and you can see it in a lot of different manifestations that can trick you. That’s why with that work up to it, even though it’s a clinical diagnosis, sometimes a biopsy might be as equally important as the lab workup, as well, if there are other factors you’re considering in making that diagnosis.

Transcript edited for clarity