Pediatric Dermatology

Transcription

1 Pediatric Dermatology Emergencies & Urgencies Nicholas V. Nguyen, M.D. Director, Pediatric Dermatology

2 Disclosures In the past 12 months, I have had the following financial relationships with the manufacturer(s) of any commercial product(s) and/or provider(s) of commercial service(s) discussed in this CME activity: Pfizer Paid Speaker/Consultant I do intend to discuss an unapproved/investigative use of a commercial product/device in my presentation

3 Learning Objectives To recognize pediatric dermatologic conditions that present emergently or urgently To identify dermatologic conditions requiring immediate action to prevent life-threatening or function-altering complications To understand the management of such conditions

4 Case 1 15-month-old female in urgent care CC: worsening rash ROS+ for rhinorrhea, conjunctivis, mild cough Amoxicillin (day 7/10) Temp 38.5 C Child appears non-toxic

5 Case 2 2-year-old female in ED CC: rash and refusal to walk Recently diagnosis of otitis media; on amoxicillin (day 10/10) Temp 39.3 C Fussy Cervical and inguinal adenopathy

6 Keys to Clinical Diagnosis Morphology Distribution HISTORY

7 Acute Annular Urticaria AKA urticaria multiforme* Cutaneous hypersensitivity reaction to viral antigenic trigger Annular variant commonly seen in infants /young children Annular, arcuate, polycyclic red wheals with ecchymotic center Lesions are MIGRATORY Associated findings: +/- Facial and acral angioedema Dermatographism Preceding viral illness (rhinorrhea, cough, diarrhea) Pruritus Shah KN, et al. Pediatrics 2007;119;e1177

8 Acute Annular Urticaria Treatment H1-blocking antihistamines Non-sedating in AM (cetirizine or fexofenadine) Sedating in PM (diphenhydramine or hydroxyzine) +/- H2-blockers Corticosteroids for severe cases not responding to antihistamines +/- Referral to allergy

9 Acute annular urticaria Misdiagnosed as EM or SSLR Dusky center mimics targetoid lesions of EM Weston JA, Weston WL. Pediatrics 1992;89:802

10 Serum sickness-like reaction (SSLR) A drug reaction characterized by urticarial skin changes, fever, arthralgia and adenopathy 1-3 weeks after drug exposure NOT true serum sickness Circulating immune complexes, hypocomplementemia, vasculitis, nephritis and proteinuria not present Young children Morphology identical to annular urticaria Potential drugs Penicillins Beta-blockers Buproprion Cephalosporins (Cefaclor) Tetracyclines Sulfonamides

11 Urticaria vs. SSLR Acute annular urticaria Lesions move within 24 hours (migratory) Total duration of rash: 2-12 d SSLR Lesions persist days to weeks (fixed) Total duration of rash: 1-6 wk Dermatographism (+) Dermatographism (-) Angioedema (++) Angioedema (+) Low-grade fever Pruritus Sometimes drug-induced High grade fever Pain (Myalgias, arthralgias) Always drug-induced

12 SSLR: treatment Discontinue the offending drug Symptoms subside in 2-3 weeks Antihistamines for mild disease Systemic corticosteroids for moderate to severe disease Prednisone 1-1.5mg/kg x 2-3 weeks

13 Case 3 13 year old male in ED CC: blisters on skin and mouth pain History of herpes labialis, last cold sore 2 weeks ago Afebrile Child appears uncomfortable when speaking

14 Case 4 12 year old female presents to ED CC: fever, cough and rash 5-6 day history of low grade fever, malaise, cough and sore throat Meds: Tylenol, ibuprofen Temp 38.9 C; appears uncomfortable

15 Case 5 15 year old female presents to ED CC: mouth sores and rash 5-6 day history of low grade fever, malaise Meds: lamotrigine for bipolar disorder, dose increase 2 weeks ago Temp 38.9 C; Pulse ox 88%; appears ill

16 Erythema Multiforme, Stevens Johnson Syndrome, Toxic Epidermal Necrolysis An Evolving Classification System EM SJS TEN

17 Erythema Multiforme Self-limited, mucocutaneous reaction Typically precipitated by preceding HSV infection Less common triggers: EBV, ORF, histoplasmosis Does NOT progress on to Stevens-Johnson syndrome Uncommon in younger children

18 Erythema Multiforme Characteristic evolution of skin lesions: Fixed, red papules => annular, iris or vesicular lesions over several days Lesions symmetrically distributed, typically acral, including palms and soles and extensor extremities Oral erosions in ~50%; other mucosal sites typically not involved Oral erosion more discrete/localized

19 Erythema Multiforme Eruptions last 2-4 weeks Recurrence common Prophylactic acyclovir or valacyclovir Symptomatic treatment Antihistamines, topical steroids, analgesics to relieve itch and discomfort

20 Erythema Multiforme is a Distinct Entity EM SJS TEN

21 Stevens-Johnson Syndrome A severe, life-threatening hypersensitivity reaction to drug or infection Prodrome of fever, malaise, headache, sore throat, rhinorrhea, cough Acute onset of mucocutaneous disease 1-14 days later 2 mucous membranes! Systemic, multi-organ disease

22 SJS / TEN? Disease Skin detachment Skin lesions* SJS <10% Macules, atypical flat targets SJS-TEN 10-30% Macules, atypical flat targets TEN >30% Macules, atypical flat targets *Typical target lesions are seen in erythema multiforme, now considered distinct from SJS / TEN

23 SJS / TEN: Pathogenesis DRUGS Typical exposure begins 7-21 days prior INFECTIONS Particularly Mycoplasma pneumoniae

24 SJS / TEN: Associated drugs Proven drugs in kids Sulfonamide antibiotics Anticonvulsants Carbamazepine Lamotrigine Phenobarbitol Possible drugs in kids Acetominophen NSAIDs Valproic acid Others* Phenytoin Allopurinol Nevirapine Oxicam NSAIDs Corticosteroids Other anti-infectives *From adult literature and case reports Levi N, et al. Pediatrics 2009;123:e297

25 SJS / TEN: Treatment Treat underlying infection if identified Stop the drug! Intensive, supportive care (burn center) Nutritional support Pain control Ophthalmology consult, daily eye care Ocular complications occur in 40% Consider amniotic membrane grafting Urology/OB GYN Corticosteroids, IVIG

26 IVIG for TEN: proposed mechanism Diagram from Metry et al, Pediatrics 2003;112:

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28 EM vs SJS/TEN SJS/TEN Drug or Infection-Induced Painful/dusky patches; bullae; atypical targets Lesions haphazardly distributed EM Hypersensitivity reaction to HSV True targets Acral distribution (palms, soles, extensor extremities 2 or more mucous membranes Oral mucosal involvement 50% Oral mucosa: diffuse necrosis/sloughing Significant Systemic Manifestations Oral mucosa: discrete/localized lesions Mild Systemic Manifestations

29 Mycoplasma Pneumoniae-Induced SJS? Adult SJS/TEN (drug), pediatric TEN (drug), pediatric SJS (infection or drug) Mycoplasma-associated SJS versus drug induced SJS Prominent mucositis Limited cutaneous involvement Exceedingly low mortality rate Lower rate of sequelae from mucosal injury High recurrence rates with subsequent infections Canavan TN et al. J Am Acad Dermatol. 2015;72(2):

30 Mycoplasma Pneumoniae-Induced SJS? Canavan TN, et al. Mycoplasma-pneumoniae-induced rash and mucositis as a syndrome distinct from Steven s Johnson Syndrome and erythema multiforme: a systematic review. J Am Acad Dermatol. 2015

31 Erythema Multiforme, Stevens Johnson Syndrome, Toxic Epidermal Necrolysis An Evolving Classification System EM Mycoplasma-associated Rash and Mucositis SJS TEN

32 Case 6 3 week old male in urgent care Seen 2 days ago by PMD for drainage from umbilical cord stump Pt awoke with raw, red skin this AM Temp 38.8 C Fussy and uncomfortable

33 Staph scalded skin syndrome Exfoliative skin disease caused by epidermolytic toxin-producing S. Aureus Neonates, infants, young children

34 SSSS: clinical findings Starts as localized pyogenic infection of conjuctivae, nares, perioral skin, perineum, umbilicus Progresses to redness with superficial erosions (periorificial face & intertriginous) Skin pain Associated fever, malaise, fussiness, poor feeding

35 Amagai M. J Am Acad Dermatol 2003;48: 244

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37 SSSS: treatment Admission to monitor fluid / electrolyte balance, temperature stability Culture pyogenic skin foci, conjunctivae, nasopharynx Treat underlying infection 1st choice: Cephalosporin or Clindamycin GENTLE skin care Bland ointment and / or non-adherent dressings Pain control