Clinical pearls in pediatric dermatology

Pediatric dermatology is a rewarding area of special interest for dermatologists. Overlapping conditions, the need for extra-gentle skin care, patient compliance, and medication adherence all present unique challenges for pediatric dermatologists. In part one of our discussion, Kelly Cordoro, M.D., associate professor of dermatology and pediatrics at the University of California in San Francisco, discusses differentiating diseases and treatment recommendations with Dermatology Times editorial advisor, Elaine Siegfried, M.D.

Elaine Siegfried, M.D.: What inspired your career choice in pediatric dermatology?

Kelly Cordoro, M.D.: My career choice in pediatric dermatology actually came as somewhat of a surprise to me. When I was a dermatology resident at University of Virginia, I really saw my future as a general academic dermatologist who wanted to incorporate kids into my practice. To dive a little bit further into that, as a dermatology resident, I spent a month with Ilona Frieden, M.D., professor of dermatology and pediatrics at UC San Francisco, to expand my knowledge and experience with kids, because we didn’t have a pediatric dermatologist at the University of Virginia. That month actually turned out to change the course of my career in full.

Check out part two of this three part series: Pediatric psoriasis, eczema:Triggers and therapies

I was quite inspired. I returned to UVA and joined the faculty there. Two years later, I moved to San Francisco to do a pediatric dermatology fellowship! I was completely sold.  The diseases are fascinating, the scope is broad, the patients are complex, and really the extra challenges of understanding skin disease in the context of rapidly evolving human beings with a different physiology as well as different developmental and psychosocial dynamics at various ages was really intellectually compelling to me. I have been a pediatric dermatologist ever since and I have not left UCSF after my fellowship.

READ: Childhood skin disease has unique challenges

Dr. Siegfried: Can you talk about your career prior to medical school?

Dr. Cordoro: Before I went to medical school, I spent two years as a research associate for Dr. David Skoner, an allergist/immunologist at Children's Hospital of Pittsburgh. So I did have a foray into pediatrics and into the research aspects of disease, but I found myself profoundly fascinated with the skin manifestations of all of these asthma patients that were coming into our clinical trials. I knew I wanted to go to medical school and pursue this interest further.

Dr. Siegfried: Is that what inspired your special interest in severe inflammatory skin disease?

Dr. Cordoro: I really don’t have an explanation for my interest in severe disease other than to say that this is the disease group that I was really organically drawn to the most. I feel most excited by and intellectually curious about this group of patients. I think the best way to summarize it is to say that if I could only see one subset of patients for the rest of my career, I would let my clinic fill with psoriasis, pityriasis rubra pilaris, connective tissues disease, graft-versus-host disease, and the like.

NEXT: Distinguish between eczema, psoriasis, atopic dermatitis and contact dermatitis?

Dr. Siegfried:I am sure you see lots of eczema, psoriasis, atopic dermatitis and contact dermatitis. How do you distinguish between all of these diagnoses?

Dr. Cordoro: Distinguishing between atopic dermatitis and psoriasis in very young and actually even older children can be really difficult. The two often overlap. In my observation, sometimes I find myself diagnosing eczematous psoriasis or psoriasiform eczemas, really a hybrid between those two diagnoses. Like you - you’re an expert clinician - I’ll try to use cutaneous clues to point me one way or another. Details about lesional morphology; distribution; special sites; what’s the overall state of the skin; are they widely xerotic or are they not; is there gluteal involvement? And if the family history and medical context and so forth doesn’t help me, I really don’t routinely perform biopsies to sort it out, because fortunately the basic management approach is similar for both conditions. Certainly atopic dermatitis far exceeds the prevalence of psoriasis but sometimes I do find it to be very challenging to disentangle the two, and I am not sure how relevant it is at the earliest stages in the youngest kids.

READ: Probiotics for healthy skin

Dr. Siegfried: Would you agree that contact dermatitis often complicates atopic dermatitis and psoriasis?

Dr. Cordoro: Absolutely. I think differentiating atopic dermatitis from contact dermatitis is one of the most difficult clinical problems I face. Oftentimes I just allow some intellectual flexibility to think there is probably an overlap here, I’m going to treat for a period of time as I would for atopic dermatitis, and then if it evolves in a characteristic way or doesn’t respond to treatment, I’ll pursue patch testing if I remain suspicious for contact dermatitis.

Check out part two of this three part series: Pediatric psoriasis, eczema:Triggers and therapies

Dr. Siegfried: For kids whose primary skin disease you think is more psoriasis than eczema, do your initial treatment recommendations differ?

Dr. Cordoro: I think the only significant difference initially is that I’ll spend a lot more time on the importance of the concepts of gentle skincare, the skin barrier and preserving and restoring the barrier in kids with atopic dermatitis. I think that’s the significant difference. In terms of selecting a treatment regimen, it’s very similar with a few exceptions - like including a vitamin D analog for psoriasis that we wouldn’t use for atopic dermatitis. With every child that I treat with psoriasis I discuss the importance of keeping the skin hydrated with a good emollient to prevent trauma and friction which prompts spread of disease via koebnerization.

If there’s overlap, I think the more complicated conversation to have with parents is explaining the natural history of two diseases. Sometimes it is difficult to get parents to understand the reason that I can’t be specific right away and the possibility of an evolving treatment plan. That can be tricky when you are trying to earn parent’s trust as a clinician.

NEXT: Use of topical corticosteroid for kids

Dr. Siegfried: Do you use topical corticosteroid monotherapy for kids who present with mostly psoriasis or exclusively psoriasis?

Dr. Cordoro: Yes. Topical steroids are absolutely the leading treatment for psoriasis and atopic dermatitis. I start with a higher potency first. I don’t start low and build up. I try to get the child clear or near clear first and then titrate the potency depending on the extent, distribution and severity. Then I introduce more options, such as vitamin D analog on the weekends, or doing a vitamin D analog in the morning and the evening, or maybe introducing a topical calcineurin inhibitor.

Dr. Siegfried: How do you feel about patch testing children?

Dr. Cordoro: I think it’s fraught with problems, it’s hard to get enough surface area to clear to even patch test, and I think in the beginning, even if you are faced with allergic contact dermatitis, the goal no matter what is to treat and clear as best we can.

Check out part two of this three part series: Pediatric psoriasis, eczema:Triggers and therapies

I have been very lucky. UCSF has a really rich tradition of expertise in patch testing with Dr. Howard Maibach, M.D., and now our newest faculty member, Nina Botto, M.D., who came to us from Tufts University.  I refer the kids for whom I am most suspicious and just cannot get them clear for any period of time or at all.

READ: The Irregular Border

Dr. Siegried: For kids who have psoriasis or eczema or inflammatory skin disease and who are using topical treatment only, how do you monitor medication adherence?

Dr. Cordoro: I don’t have the tricky tubes and jars that monitor how many times the lid has been taken off that Dr. Steven Feldman made famous in his studies on topical compliance. So for the lack of having trickery up my sleeve, I really just follow clinically. I think the most important thing is to assume that the prescribed therapeutic regimen is adequate to manage the different components of the disease. I assume that if I have given the right regimen and they are not responding, then there is either an overlapping factor that I missed and need to address or they’re not compliant.

READ: Part two of Pediatric Dermatology

I think the other clinical pearl that I have learned over time is that we really need to consistently review what is actually being used and how it’s being used to assure that what we have prescribed has been translated into the proper use of that agent. These regimens are not straightforward, and what the patient is actually using and how are they using it may not even closely resemble what we recommended. Medication adherence and compliance is really tricky because there are so many moving parts, and that’s part of the fun and challenge of it all.

NEXT: 30% error in pharmacy dispensing, compared to prescribed

Dr. Siegfried: We always ask our patients to bring in their tubes and have actually found about a 30% error in pharmacy dispensing, compared to what we have prescribed, either in the quantity, vehicle or the product concentration.  The labels on the tubes sometimes don’t represent what’s in the tube that the label had been attached to.

Dr. Cordoro: That’s something I haven’t even considered as a possibility. Yet another challenge.

Dr. Siegfried: The effort required to monitor the actual medication and quantities used can be a nightmare. I would say 70% of people probably don’t use adequate amounts and maybe 20% use too much. I rarely give refills. I prefer that the patient call their pharmacist to fax the refill request, so we know when they get their refills. Monitoring adherence is an important but difficult problem. There’s no right answer.

Check out part two of this three part series: Pediatric psoriasis, eczema:Triggers and therapies

Dr. Cordoro: I think one of the challenges also is the psychological warfare that we play when a patient gets a prescription. For example, if we write “450 gram tub” for very widespread, severe disease, and the pharmacy supplies only a 60 gram tube, psychologically, a parent becomes concerned about how much to use given the limited quantity supplied.  At the pharmacy, patients are often given advice, for example, told not to use the prescription for more than two weeks; parents are told that they shouldn’t be using the prescription on their 5-year-old child, etc., but they wait for three months to come back to tell you that.

Earlier in my career I became frustrated, even angry, when I would hear this, and I would call pharmacies and rant and rave. I think a lot of these really complex inflammatory disease patients aren’t really getting the time that they need from the doctor in that regard. We need to really educate them that we are skin experts who are prescribing the medication this way for a reason, because once they leave your clinic, they are open to the world, and I think that’s when modifications happen. It’s a fascinating and frustrating problem.

READ: Nailing the diagnosis

Dr. Siegfried: We have a number of problems with very limited formularies. Do you face that challenge?

Dr. Cordoro: Absolutely, all the time. I will say this: I feel very fortunate that one of the lessons I learned very early in my residency at the University of Virginia was to be cost-conscious. I, to this day, rarely will write for branded medication if there is a suitable generic alternative. And I think this actually plays into my favorite part of dermatology: The art of mastering the treatment of skin diseases and the ability to find alternate regimens and generic regimens that work, as well as compounding.

If we can get a pharmacist to make a cream for diaper dermatitis with inexpensive ingredients by mixing a little hydrocortisone, a little Nystatin, and a little zinc oxide, you can save the patient anywhere from $10-$25 which is the price of the commercially available brand. Compounding is becoming a lost art really.

Check out part two of this three part series: Pediatric psoriasis, eczema:Triggers and therapies

I think one of the other tricks to dealing with these formulary restrictions is to ask patients to compound simple things by themselves. I will often have a patient add a tube of a topical steroid into plain white petrolatum, or another emollient, to create something that will last them a bit longer, give them a little anti-inflammatory action while repairing the barrier. I think “skin devices”, while well-intentioned, are not affordable for most of my patients and they won’t be covered.

Formulary problems are also difficult with systemic medications. If there is a systemic medication to be prescribed and I don’t believe there is an adequate substitute, I will call the carrier personally to provide the medical rationale and seek approval for its use.

My UCSF staff are incredibly gifted in getting these prior authorizations for necessary medications. Sadly this has become just a routine practice for so many prescriptions. Even now, generics require prior authorization for medications that we used to just prescribe liberally.

NEXT: Getting kids access to medication is increasing

Dr. Siegfried: Yes. The amount of time that it takes us to get kids access to medication is increasing - particularly for the topical calcineurin inhibitors. Having a generic alternative helps, but there are increasing requirements for step edits and prior authorization.

Dr. Cordoro: I think something that is profoundly informative is looking at the patient-visit-to-telephone-call ratio. So for the ones who call a lot, we are starting to see with a lot of different medications that, for example, we have one patient visit for atopic dermatitis and nine to 12 phone calls about authorization issues and pharmacy issues. We have not done this formally, but this was just an observation we have recently been making.

Check out part two of this three part series: Pediatric psoriasis, eczema:Triggers and therapies

Where do we draw the line between our ability to give excellent care and the lack of ability for the patients to get the medications, and then the staff time and money wasted and time wasted to get the medicines. It’s just becoming so complex and problematic.

I think we need this kind of data to show companies and carriers that this is just ridiculous when physicians are spending this type of time on these kinds of problems.

READ: Pediatric trials for AD systemic treatments

Dr. Siegfried: What about for kids less than two years old, for example, who need a steroid-sparing agent and they can’t get a topical calcineurin inhibitor, what do you do?

Dr. Cordoro: I used to have no problem prescribing and fighting for prior authorization, but when the Food and Drug Administration put the black box warning on the topical calcineurin inhibitors, it just gave us all pause. Even though many of us don’t believe it’s medically valid and it was based on a theoretical risk, carriers just absolutely use that to their advantage to refuse the medication.

I will call sometimes, but we were very unsuccessful at getting this medication for kids less than two. We’re just unsuccessful in getting topical calcineurin inhibitors. So, oftentimes I lower the potency of the topical steroid or try to find an alternative like tar. Tar is a big workhorse in my clinic for both psoriasis, atopic dermatitis, and even seborrhea. So small amounts, for example, of liquor carbonis detergens (LCD) 3% or so mixed in petrolatum. There was some back and forth about coal tar as a cutaneous carcinogen several years ago. There have been wonderful studies done about tar for clinical use in dermatology out of the Mayo Clinic. Their 25-year long-term follow-up study¹ showed no increased risk of cutaneous carcinoma with tar combined with UV light, so I think the data and efforts like that helped California to keep tar as a therapeutic option.  

Dr. Siegfried: How do you handle steroid-phobia?

Dr. Cordoro: My approach has evolved over the years and really varies from patient to patient and family to family. I consider the overall context of the condition and what the parents are telling me. I try to read into what their motivations are and what their beliefs are. I think there are probably two main types of families that I see: There are parents who have been misinformed. They want detailed explanations of pathophysiology of atopic dermatitis, the history of steroids, and the mechanism of action of topical steroids or topical calcineurin inhibitors. Once they understand the mechanism and the rationale for the use, they’re fine with using these therapies.

Check out part two of this three part series: Pediatric psoriasis, eczema:Triggers and therapies

The second type of families I see consist of parents that have a fixed false belief about these medications and they just absolutely do not want to consider their use. These are the ones that need to hear simply that these approaches are the gold standard and widely accepted by Western-trained dermatologists and that we vet these therapies through data analysis and expert consensus.

READ: Part two of Pediatric Dermatology

When I talk to patients about the fact that these medications have been formalized in the therapeutic guidelines by the American Academy of Dermatology, this helps.

I have written about this for parents and families because it’s such an issue.² I often print this article and hand parents a copy. I also tell parents that I am more concerned that they won’t use these topical agents than I am that they will. For the right parent, that resonates. I adopted that approach from Ilona Frieden, and it is very effective.

The message is that the parent has a child with this difficult skin condition who is missing school, miserable, has dropping grades, the parents are missing work, they are regularly cleaning bloody sheets; so what is the rationale to withhold the medication? There is a risk of untreated disease that outweighs the risks of treatment, and that’s what I try to communicate. I think for some parents you get through, and for others you will never win the battle.

Check out part two of this three part series: Pediatric psoriasis, eczema:Triggers and therapies

Dr. Siegfried: Do you have any sound bites for black box topical calcineurin inhibitor phobia?

Dr. Cordoro: I always tell patients that there is a black box warning and I describe exactly what the truth is in lay terms: The medication was fed to primates in high quantities of active ingredient, some of the primates developed lymphomas. I mention that the warning is based on a theoretical risk and the data derives from nonhuman subjects. I mention that we’re making efforts to get this black box warning overturned. And I give the patient the choice. 

NEXT References

References

1. Pittelkow MR, Perry HO, Muller SA, Maughan WZ, O'brien PC. Skin cancer in patients with psoriasis treated with coal tar. A 25-year follow-up study. Arch Dermatol. 1981;117(8):465-8.

2. Topical Steroid Use and Children: Reasonable Fear or a Phobia? http://www.skinsight.com/info/blog/2011/06/15/topical-steroid-use-and-children-reasonable-fear-or-phobia

In August, Dr. Cordoro will continue this discussion, addressing triggers and systemic treatments.