Hot Seat Case #96: 11 yo F with leg pain and rash
Posted on: June 15, 2017, by : Haroon Shaukat MD
Haroon Shaukat, MD Children’s National Medical Center
with Mike Quinn, MD Children’s National Medical Center
11 yo female with PMH of asthma, obesity, and seasonal allergies who presented with 1 day of bilateral lower extremity pain, edema, and rash. Mother stated she was walking around all day on a field trip at the museum the day prior to arrival. On the morning of presentation, she awoke with bilateral lower extremity pain and swelling. She stated it was hard to walk or bear weight. The rash was nonpruritic, started on her feet, and had already spread up her legs towards her thighs. She denied any recent travel, change in detergents, lotions, soaps, insect bites, wounds, or trauma. She reported that 2 weeks ago, she had swelling in her feet after playing sports, but this resolved after soaking her feet in Epsom salt.
ROS:
(+) Peripheral edema, 30 lb intentional weight loss this past year, Muscle pain, Rash.
(-) No fever, No conjunctivitis, No sore throat, No dizziness, No syncope, No chest pain, no shortness of breath, No abdominal pain, dysuria, hematuria.
Family Hx:
MGM – Asthma, PGM – DM and HTN, Denied autoimmune or rheumatologic diseases.
Social Hx:
Denied drug use or sexual activity.
PE: T Afebrile, HR 70, RR 20, BP 153/80, 98% on RA Wt 116 kg
General: AO x 3, Obese, Cooperative
Skin: Patchy, non-blanching, erythematous maculopapular rash on bilateral lower extremities up to shins in scattered areas, not serpiginous
HEENT: No periorbital edema, MMM, No oropharyngeal ulcers
Lungs: CTAB
Heart: RRR,S1/S2, No m/r/g, Normal peripheral pulses and capillary refill
Abdomen: Soft, NT/ND, No organomegaly
MSK: Tenderness of bilateral lower extremities from feet to shins, 2+ non pitting edema
Neuro: Motor and sensation intact, Refused to bear weight or ambulate
While you are examining the patient, the father insists that his child needs a lower extremity duplex ultrasound. He explains that he had a family member die from a pulmonary embolus.
Exam findings were not consistent with DVTs, so labs were ordered and a dose of toradol was trialed.
Labs are significant for the following:
CBC: WBC 12k, Plt 289
CMP: Albumin 3.7
CRP: 1.56
ESR: 10
CK: 493
C3: 142
UA: 1.020, negative
At this point, the patient’s pain has not changed at all from an 8/10 despite the toradol.
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A picture is worth a thousand words…a picture of this child’s rash might be helpful, especially if it looked vasculitic (microangiopathic) in nature. The patient’s BP is elevated, there is a concerning rash, elevated inflammatory markers, and fluid retention — I’d be worried about a glomerulonephritic process. Also, dermatomyositis is also on the differential.
Dermatomyositis is a great thought i didn’t think of here. She’s the right age for it. This is why it’s important to determine if she’s WEAK or just doesn’t want to walk because it hurts. I think dermatomyositis is unlikely here because she would have a much more elevated CPK and it’s usually proximal muscles (trunk) not distal ones. And shouldn’t be associated with edema. the classic rashes with dermatomyositis are facial and on the knuckles.
It’s important to approach these kind of cases and ask what really needs emergency evaluation here. Your job in the ED is not to solve the crime, but to prevent significant morbidity/mortality and intervene if there is early management that will help. There’s a lot going on here but make sure we separate what’s emergency management and outpatient. I’m not sure there’s much in this case that tells me that anything needs to be done in an ED except some vascular stuff. But this girl really needs help and some good coordinated care to make a diagnosis somewhere that’s not the ED.
A more thorough PE and history are helpful here. I would try to avoid the use of the term maculopapular rash. few rashes are actually macular and papular. This terminology is abused and i think this needs to be described better. And you need to explain why she isn’t walking. this is important. Not “wanting” to walk is not an appropriate physical exam finding. does she have weakness? is she having pain with ambulation? This evaluation is the only thing that would potentially require more of an ED evaluation and is important. I would ask about supplements if she has lost 30kg and still weighs 117kg. Sounds fishy. i worry she could be having a reaction to some type of weird herbal supplement or medication she’s too shy to disclose at triage.
I want to say the description sounds like she has erythema nodosum, although hard to tell here. I don’t think that’s usually associated with edema but unsure. I thought with erythema nodosum it just was tender over some of the lesions but not the entire lower leg or true edema. Erythema nodosum can be associated with a host of rheumatologic diseases and IBD and others that all requires some outpatient evaluation. She has systolic hypertension and is overweight and has sorta elevated inflammatory markers so she some systemic findings that require follow-up and additional history. I’m assuming the CPK is from the walking and is not that elevated.
The edema is a bit troubling. There are not that many things that cause systemic edema. Capillary leak (allergic), decreased oncotic (hypoalbuminemia), and increased hydrostatic. You’ve already proved that she doesn’t have nephrosis with a normal UA and serum albumin. It can’t really be a localized lymphedema if it’s bilateral. So that leads me to believe she has increased hydrostatic pressure which may be represented by the high systolic pressure.
So I might get an EKG and chest XR to evaluate for other signs of cardiac insufficiency (pleural effusions, etc) given her new edema and systolic hypertension. And if normal would have the patient follow-up with rheumatology assuming she could actually walk (and just doesn’t want to because of pain/edema). Might not be a bad idea to start her on a low dose ACE inhibitor or nifedipine that could help here. If it only hurts her to walk on her legs, she shouldn’t have hypertension when just sitting down. It wouldn’t be unreasonable to check BP in a few different positions to isolate if it’s a chicken or an egg. And you can make fun of me for getting bilateral US/DVT (which I would because its important to exclude and the patient has multiple risk factors, although obviously unlikely with rash and bilateral presentation).
Don’t draw lots of rheum labs from the ED. it’s a huge waste of money. If she has a follow-up plan, she have coordinated care for a blood-draw prior to her appointment.
Thanks for all that insight Dr. Mathison. Although I would disagree that drawing the rheum labs are not a waste of money in that it makes the outpatient visit more fruitful not only for the patient but the rheumatologist as well. It gives them a baseline to start with and grow upon and hopefully narrow down the ultimate differential. It also is an opportunity to obtain a trend on their favorite labs, inflammatory markers!
A d-dimer might have been a nice step for ruling out DVT in what sounds like a low risk patient before ultrasound. Of course it may be elevated if there is another inflammatory process at play, but could have made for a more efficient work up while addressing the families fears. I think it would also be reasonable to decline DVT w/u altogether and explain to parents it’s just not at all consistent with thrombosis.