SAEM Clinical Images Series: More Than Skin Deep

skin

A 57-year-old female college counselor living in the northeastern United States with no PMH presented for evaluation of rash, joint pain, and dyspnea for the past three weeks. The patient first noticed the rash on her upper back, describing it as being itchy. The rash then spread to her face, scalp, and thighs. Two weeks ago, she noticed swelling in her hands and had a gradual onset of dyspnea on exertion. The patient has pain in her hands and when moving her fingers. She denied fever, cough, chills, chest pain, headache, vision changes, focal weakness, abdominal pain, nausea, vomiting, and diarrhea. She denied recent travel, sick contacts, significant time spent outdoors, known tick bites, new medications, and changes in her diet. She has never had a rash like this before.

Vitals: BP 110/70; HR 86 BPM; RR 18 breaths/min; T 37°C; SpO2 96% on RA

Skin: Warm and dry. There is a macular, violaceous rash to the upper back and upper thighs. The patient’s hands are slightly edematous with nontender papules on the palmar aspect of the hands.

CV: Heart sounds are normal. No jugular venous distention or lower extremity edema.

Lungs: There are faint bibasilar rales heard on auscultation of the chest.

Extremities: Full ROM of the joints and there are no bony deformities. The patient does not have muscular tenderness.

Neuro: Within normal limits; muscle strength 5/5 in all four extremities.

CBC w/ differential: Normal

BMP: Na 142, K 3.8, Cl 106, HCO3 24, BUN 16, Cr 0.44, Glu 112, Ca 8.5, Mg 2.2, Phos 3.4

LFT’s: AST 105, ALT 76, ALP 68, Tbili 0.2 Alb 3.7

PT/PTT/INR: 12.0s/32.2/1.04

D-dimer: 347

ESR: 62

CRP: 0.75

Ferritin: 625

CK: 195

LDH: 276

Procalcitonin: undetectable

Amyopathic dermatomyositis – specifically anti-melanoma differentiation-associated gene 5 (anti-MDA5) positive dermatomyositis as determined by subsequent inpatient auto-immunological workup. Compared to other dermatomyositides, anti-MDA5 positive dermatomyositis is characterized by an absence of traditional muscular involvement. Additionally, patients can present with respiratory symptoms related to interstitial lung disease (ILD). One phenotype of this condition is associated with a rapidly progressive ILD, but respiratory involvement may be delayed years after the initial symptoms are noticed. The patient’s clinical images demonstrate a macular, violaceous rash in the “shawl sign” and “holster sign” distribution patterns typical of dermatomyositides. Palmar papules (not to be confused with Gottron’s papules which are found on the dorsal surface of the metacarpophalangeal and interphalangeal joints) are fairly specific for anti-MDA5 positive dermatomyositis

There are no specific guidelines for treating anti-MDA5 positive dermatomyositis. Patients are typically started on a high-dose steroid regimen. A rheumatology consult should be obtained to determine if the patient would benefit from treatment with immunosuppressants. Given her complaints of dyspnea, the patient should undergo a non-contrast CT of the chest to evaluate for evidence of scarring or pulmonary fibrosis.

Take-Home Points

  • Anti-MDA5 positive dermatomyositis is associated with rapidly progressive ILD has a poor prognosis.
  • This rare form of dermatomyositis should be suspected if the patient has respiratory complaints in addition to the hallmark cutaneous findings commonly observed in all types of dermatomyositides. Palmar papules are fairly specific for anti-MDA5 positive dermatomyositis. It often lacks typical historical and physical features of muscular weakness.
  • Treatment involves high-dose corticosteroids and consideration of immunomodulator therapy.

  • Allenbach Y, Uzunhan Y, Toquet S, Leroux G, Gallay L, Marquet A, Meyer A, Guillaud C, Limal N, Gagnadoux F, Hervier B, Borie R, Deligny C, Terrier B, Berezne A, Audia S, Champtiaux N, Devilliers H, Voermans N, Diot E, Servettaz A, Marhadour T, Castelain V, Humbert S, Blanchard-Delaunay C, Tieulie N, Charles P, Gerin M, Mekinian A, Priou P, Meurice JC, Tazi A, Cottin V, Miyara M, Grange B, Israël-Biet D, Phin-Huynh S, Bron C, De Saint Martin L, Fabien N, Mariampillai K, Nunes H, Benveniste O; French Myositis Network. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases. Neurology. 2020 Jul 7;95(1):e70-e78. doi: 10.1212/WNL.0000000000009727. Epub 2020 Jun 2. PMID: 32487712; PMCID: PMC7371381.
  • Nombel A, Fabien N, Coutant F. Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies. Front Immunol. 2021 Oct 20;12:773352. doi: 10.3389/fimmu.2021.773352. PMID: 34745149; PMCID: PMC8564476.

SAEM Clinical Images Series: Man with a Recurrent Rash

rash

A 33-year-old male presented to the emergency department with a diffuse pruritic rash that appeared several days after starting Trimethoprim/Sulfamethoxazole (TMP-SMX) for a dental infection. Initially beginning on the torso and low back, the rash spread to the palms, soles, and genitalia. Progression stopped after discontinuing TMP-SMX. He conveyed a remote history of a similar rash following use of an unknown medication, and noted that several of the current lesions arose at the same location as previous.

Skin: Widely distributed violaceous, non-blanching patches with a dusky center. Lesions ranged from 3 cm to 10 cm, and included palms and soles. There was no mucosal involvement.

Non-contributory

Fixed drug eruption (FDE). FDE is an uncommon, potentially life-threatening CD8+ T-helper cell-mediated hypersensitivity reaction to certain drugs, commonly NSAIDs, antibiotics, and antiepileptic [1].

Skin findings typically arise within two days of exposure and then more rapidly with subsequent exposures [2]. Characteristically, recurrent lesions appear at the same sites as prior lesions (hence “fixed”) but may arise in additional locations. The rash is classically divided into two phases: an acute phase of pruritic violaceous patches and plaques with central duskiness, followed by a residual phase of hyperpigmentation that can last several months. The sulfonamide moiety of TMP-SMX is a common cause of FDE [3]. Management of FDE anchors on identification and discontinuation of the causative agent. The majority of cases involve five or fewer lesions, however generalized or bullous cases (> 10% total body surface area, or involvement of 3 or more anatomic sites) [1], may require aggressive wound care and carry a mortality rate up to 22% [4]. Topical or systemic steroids are common adjuncts and there is limited evidence suggesting the utility of systemic cyclosporine for severe cases [1]. Patients need to be carefully advised on the risks of specific medication use and can expect a gradual resolution of lesions over the coming months.

Take-Home Points

  • FDE is a potentially life-threatening hypersensitivity reaction to certain drugs.
  • Recurrent lesions in similar distribution is a hallmark of FDE. Avoidance of the causative agent is the mainstay of management.
  1. Anderson HJ, Lee JB. A Review of Fixed Drug Eruption with a Special Focus on Generalized Bullous Fixed Drug Eruption. Medicina (Kaunas). 2021 Sep 1;57(9):925. doi: 10.3390/medicina57090925. PMID: 34577848; PMCID: PMC8468217.
  2. Flowers H, Brodell R, Brents M, Wyatt JP. Fixed drug eruptions: presentation, diagnosis, and management. South Med J. 2014 Nov;107(11):724-7. doi: 10.14423/SMJ.0000000000000195. PMID: 25365443.
  3. Chow TG, Khan DA. Sulfonamide Hypersensitivity. Clin Rev Allergy Immunol. 2022 Jun;62(3):400-412. doi: 10.1007/s12016-021-08872-3. Epub 2021 Jul 1. PMID: 34212341
  4. Lipowicz S, Sekula P, Ingen-Housz-Oro S, Liss Y, Sassolas B, Dunant A, Roujeau JC, Mockenhaupt M. Prognosis of generalized bullous fixed drug eruption: comparison with Stevens-Johnson syndrome and toxic epidermal necrolysis. Br J Dermatol. 2013 Apr;168(4):726-32. doi: 10.1111/bjd.12133. Epub 2013 Feb 16. PMID: 23413807.

SAEM Clinical Images Series: Wolf in Sheep’s Clothing

wolf

A 55-year-old female with a history of hyperlipidemia presents after a syncopal episode. She had mild nausea and diarrhea on the morning of presentation but otherwise had no prodromal symptoms before suddenly losing consciousness in a grocery store. Of note, she recalls a similar syncopal episode in the remote past, also preceded by gastrointestinal symptoms at that time. At present, she is symptom-free.

Vitals: BP 135/71; HR 52; Temp 98°F; RR 18; SpO2 100% on room air

General: Tired appearing

CV: 2+ peripheral pulses. Regular rate and rhythm, no murmurs, rubs, or gallops.

Pulmonary: No increased work of breathing. Lungs clear to auscultation bilaterally.

GI: Soft, non-distended, non-tender to palpation.

Non-contributory

Wolff-Parkinson-White Syndrome (WPW)

Short PR interval (< 0.12 seconds) and slowed upstroke of the QRS complex, referred to as a delta wave, which are both seen in our patient. These particular EKG findings define a “Wolff-Parkinson-White Pattern.”

WPW is a pre-excitation syndrome characterized by an accessory pathway caused by a congenital failure of cells to resorb near the AV valves. This accessory pathway conducts impulses faster than the AV node, causing a short PR interval. WPW Syndrome consists of characteristic EKG findings as well as symptomatic arrhythmias. Patients with WPW may classically present after a syncopal episode due to an arrhythmia involving the accessory pathway. Most commonly, WPW is associated with atrioventricular nodal reentrant tachycardia (AVNRT) and atrial fibrillation.

First-line treatment for WPW-mediated tachyarrhythmia consists of procainamide, which blocks conduction through the accessory pathway. An exception to this would be the hemodynamically unstable patient, who should be cardioverted. AV nodal blocking agents should be avoided in patients with tachyarrhythmias as they can cause increased conduction to the ventricles through the accessory pathway, leading to potential ventricular arrhythmias and hemodynamic instability. Ablation of the accessory pathway is indicated in those with symptomatic tachyarrhythmias and leads to successful remission in about 90 percent of cases.

Take-Home Points

  • The WPW pattern on EKG consists of a short PR interval and a delta wave.
  • Patients with WPW Syndrome classically present with symptomatic arrhythmias (including syncope) and EKG findings consistent with WPW pattern.
  • The most common arrhythmias seen in WPW include AVNRT and atrial fibrillation, which should be managed with procainamide. Avoid the use of AV nodal blocking agents.

  • Conover MB. Diagnosis and management of arrhythmias associated with Wolff-Parkinson-White syndrome. Crit Care Nurse. 1994 Jun;14(3):30-9; quiz 40-1. PMID: 8194348.
  • Dagres N, Clague JR, Kottkamp H, Hindricks G, Breithardt G, Borggrefe M. Radiofrequency catheter ablation of accessory pathways. Outcome and use of antiarrhythmic drugs during follow-up. European heart journal. 1999 Dec 1;20(24):1826-32.
  • Wolff L, Parkinson J, White PD. Bundle-branch block with short P-R interval in healthy young people prone to paroxysmal tachycardia. 1930. Ann Noninvasive Electrocardiol. 2006 Oct;11(4):340-53. doi: 10.1111/j.1542-474X.2006.00127.x. PMID: 17040283; PMCID: PMC6932258.

By |2023-11-12T13:55:35-08:00Nov 6, 2023|Cardiovascular, ECG, SAEM Clinical Images|

ALiEM AIR Series | Trauma 2023 Module

Welcome to the AIR Trauma Module! After carefully reviewing all relevant posts in the past 12 months from the top 50 sites of the Digital Impact Factor [1], the ALiEM AIR Team is proud to present the highest quality online content related to related to trauma in the Emergency Department. 8 blog posts met our standard of online excellence and were approved for residency training by the AIR Series Board. More specifically, we identified 3 AIR and 5 Honorable Mentions. We recommend programs give 4 hours of III credit for this module.

AIR Stamp of Approval and Honorable Mentions

In an effort to truly emphasize the highest quality posts, we have 2 subsets of recommended resources. The AIR stamp of approval is awarded only to posts scoring above a strict scoring cut-off of ≥30 points (out of 35 total), based on our scoring instrument. The other subset is for “Honorable Mention” posts. These posts have been flagged by and agreed upon by AIR Board members as worthwhile, accurate, unbiased, and appropriately referenced despite an average score.

Take the AIR Trauma Module at ALiEMU

Interested in taking the AIR quiz for fun or asynchronous (Individualized Interactive Instruction) credit? Please go to the above link. You will need to create a free, 1-time login account.

Highlighted Quality Posts: Trauma

SiteArticleAuthorDateLabel
Rebel EMTrauma Resuscitation UpdateSalim Rezaie, MDMay 25, 2023AIR
EM DocsUnstable Pelvic Trauma PatientLuke Wohlford, MDJune 3, 2023AIR
EM DocsMaxillofacial TraumaForrest Turner, MDOctober 17, 2022AIR
Rebel EMPATCH trauma trialAnand Swaminathan, MDJune 19, 2023HM
Don’t Forget the BubblesBlast InjuriesAndrew Tagg, MDMarch 16, 2023HM
Don’t Forget the BubblesPenetrating Chest TraumaSarah Davies, MD and Kat Priddis, MDJuly 1, 2023HM
St Emlyns BlogRefresher on Blood Transfusion in TraumaRichard Carden, MDApril 13, 2023HM
RCEMlearningBlast InjuriesAlison Tompkins, MDJune 30, 2023HM

(AIR = Approved Instructional Resource; HM = Honorable Mention)

If you have any questions or comments on the AIR series, or this AIR module, please contact us!

Reference

  1. Lin M, Phipps M, Chan TM, et al. Digital Impact Factor: A Quality Index for Educational Blogs and Podcasts in Emergency Medicine and Critical Care. Ann Emerg Med. 2023;82(1):55-65. doi:10.1016/j.annemergmed.2023.02.011, PMID 36967275

ACMT Toxicology Visual Pearl: Who Doesn’t Like a Nice Rosé?

test tube of different color urine

What clinical presentation and medication is associated with this urinary discoloration?

  1. Critical illness requiring intubation and propofol sedation
  2. Cyanide toxicity requiring sodium thiosulfate
  3. Iron toxicity treated with deferoxamine
  4. Refractory vasoplegic shock treated with methylene blue
  5. Septic shock treated with vancomycin and cefepime

(more…)

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