Objectives Dermatologic Emergencies: What’s That?

• Identify, appropriately work-up, and stabilize urgent/emergent dermatologic conditions • 3 cases that illustrate: – – – – – – –

Erin Mathes, MD Assistant Professor Dermatology and Pediatrics UCSF

Erythema Multiforme Stevens-Johnson Syndrome Toxic Epidermal Necrolysis Staph Scalded Skin Syndrome Eczema herpeticum Eczema coxsackium Staph superinfection

I have no relevant conflicts of interest.

How to describe what you are seeing... over the phone

Clues: When to Worry • Age (newborn and young infants) • High fever, toxicity • Morphology

• A picture is worth a thousand words • Extent: What body surface area is involved? – the patient’s palm = 1%

– blistering, mucosal involvement, hemorrhage

• Specific medications – anti-convulsants, antibiotics, NSAIDS

Case 1: This 5 yo boy with a seizure disorder and language delay has had fever, malaise, lymphadenopathy and a sore throat for 4 days. He has been taking Tylenol for 4 days, and lamotrigine for 6 weeks. What is the most likely diagnosis?

Case 1: 5 yo with sz d/o and rash. What is the most likely diagnosis? 1. 2. 3. 4.

Erythema multiforme Kawasaki Disease Stevens-Johnson syndrome (SJS) Vasculitis

Case 1

What is SJS? • Severe, life-threatening mucocutaneous disease • Clinical syndrome - no definitive diagnostic test

SJS vs EM vs TEN

• atypical “targetoid” lesions, fragility, denudation ~10%BSA • ≥ 2 mucous membranes (mouth, eyes) • systemic signs: fever, respiratory symptoms

The SJS Spectrum

Erythema Multiforme Minor and Major

Stevens Johnson (SJS) < 10% BSA

The SJS Spectrum

Erythema Multiforme Minor and Major

SJS-TEN overlap

10-30% BSA

Stevens Johnson (SJS) SJS-TEN overlap

< 10% BSA

Toxic Epidermal Necrolysis (TEN)

10-30% BSA

Toxic Epidermal Necrolysis (TEN)

> 30% BSA

> 30% BSA

Infection Low Mortality

Drug High mortality

EM vs SJS vs TEN Rash

EM

EM major

SJS

SJS-TEN

TEN

Typical targets

Typical targets

Dusky red, atypical targets, Detachment

Dusky red, atypical targets, Detachment

Poorly delineated dusky plaques, large sheets of detachment

30% with spots >10% without spots

BSA Detached

Bolognia. Dermatology. 2nd Edition.

Distribution

Extremities, face

Extremities, face

Trunk, face (+confluence)

Trunk, face (++confluence)

Face, trunk, ext (+++confluence)

Mucosal Involvement

None, mild

Severe

Severe

Severe

Yes*

Systemic Symptoms

Absent

Usually

Usually

Always

Always

Progression to TEN

No

No

Possible

Etiology

HSV, other infectious

HSV, mycoplasma, rare drug

Drug Mycoplasma HSV

Drug

Drug

SJS: Causes

Stevens-Johnson Syndrome (SJS) (Mycoplasma Associated)

• DRUGS – – – –

Many drugs implicated Anticonvulsants > antibiotics > NSAIDs Typically 7-21 days after start Drugs with longer half-lives more likely to cause a fatal reaction

• Mycoplasma – up to 25% of pediatric patients with SJS – more mucosal, less skin, +cough

• HSV • Unknown

Why isn’t this EM?

Erythema Multiforme • Target lesions with 3 zones • Dusky center • pale edematous ring • peripheral erythematous margin

• Discrete lesions • Usually no/mild systemic signs

Variety of targets in EM

Bolgnia, Dermatology, 2nd ed.

Erythema Multiforme

EM vs SJS

It looks like EM now, but… • Be more worried if you see: – Atypical targets – Trunk > Acral lesions – Confluent skin lesions – Bullous skin lesions – Continuing rapid progression

Typical targets EM

Atypical targets SJS

TOXIC EPIDERMAL NECROLYSIS

Why isn’t this TEN?

TEN with spots >30% BSA detached TEN without spots >10% BSA detached

TEN = Full thickness skin necrosis Shiny dermis underneath

SJS Initial Management & Work-Up • • • • • •

ABCs Stop the causative drug (and all non-essential drugs) Admit to ICU or burn unit if >10-20% BSA Call dermatology/ophthalmology/urology Labs: CBC, Lytes, BUN, Cr, LFTs Check for Mycoplasma, HSV (IgM)

Practical Treatment SJS Supportive Care

EM

SJS

TEN

• Meticulous daily wound care – Wash with saline, gently remove crust around orifices – Provide suction for secretions – Cover denuded areas (& corners of mouth) with vaseline gauze – Pressure bed – Avoid friction, trauma – Reverse isolation

•Treat infection •Steroids?

• Surveillance cultures (?)

•Stop drug •Treat Infection •Early steroids •IVIG?

•Stop drug •IVIG

• Hydration (careful not to overload) • Nutrition (NG)

What is going to happen to this child?

Outcome of SJS/TEN spectrum

Finkelstein Y, et al. Pediatrics. Sept 2 2011

Outcome of SJS/TEN spectrum Case 2: An 8 year old otherwise healthy boy presents with a 2 day history of an acute-onset, progressive blistering eruption associated with skin pain, malaise, and low grade fever. He is mildly tachycardic, but other VS are stable. Which of the following is the most likely diagnosis?

Finkelstein Y, et al. Pediatrics. Sept 2 2011

Case 2: 8 yo with blistering. Diagnosis? 1. 2. 3. 4.

Kawasaki Disease Staph Scalded Skin Syndrome Toxic Epidermal Necrolysis Toxic Shock Syndrome

SSSS • Begins as a localized, often occult infection – – – – – – – –

Nasopharynx Perioral Conjunctiva Umbilicus Paronychia Wound Urine Middle Ear

Case 2 SSSS vs TEN vs TSS

SSSS: Etiology • Staph produces an exfoliative exotoxin • Exotoxin cleaves desmoglein 1  superficial epidermal cleft, acantholysis

• Progresses to generalized erythema and skin fragility

Staphyloccal Scalded Skin Syndrome

SSSS

• Clinical Presentation – Neonates: Widespread erythema, superficial erosions – Toddlers & Children: erythema, periorificial scale and erosions, skin fragility and pain – Adults: rare - protective antitoxin

Source: blistering dactylitis & conjunctivitis

Perioral furrowing, scale Skin pain

Why isn’t this TEN?

Why isn’t this TEN?

Not shiny = SSSS Superficial epidermal split

Why isn’t this toxic shock syndrome?

Shiny = TEN Subepidermal split

Toxic Shock Syndrome • Rarely a primarily cutaneous disease • Staph produces superantigens that cause: – – – –

SSSS: Management • • • •

Admit (especially in younger pts) Dermatology consult Culture potential sources Empiric anti-staph antibiotics (cover for MRSA) +/- Clindamycin – Clindamycin inhibits toxin production – d/c with abx based on cx results

• Careful FEN management • Pain management

fever rash hypotension organ system involvement

Case 3: A 13 yo girl with a history of atopic dermatitis presents with 1 day of a new rash around her eyes and mouth, and low grade fever. What is the best diagnosis?

Case 3: Best Diagnosis? 1. 2. 3. 4.

Contact Dermatitis Eczema coxsackium Eczema herpeticum Staph superinfection

Eczema Herpeticum • Disseminated HSV in pts with chronic skin dz • Abrupt onset fever, malaise • Painful • History of HSV exposure or prior infection • Delay in Dx common

Eczema Herpeticum: Morphologic Clues • Monomorphous erosions > vesicles • Lesions favor – Areas of active dermatitis – Head, neck & trunk

Eczema Herpeticum vs. Contact Dermatitis

Eczema Herpeticum vs. Staph Superinfection

Eczema Herpeticum vs. Eczema Coxsackium

Strep Superinfection

Eczema Herpeticum Treatment • • • •

Culture, DFA or PCR Culture for bacteria Ophthalmology consult (for periocular involvement) Dermatology consult

• Prompt high dose acyclovir • Empiric antibiotics if signs of bloodstream infection • Topical steroids okay • Avoid systemic steroids

Eczema Herpeticum Sequelae • • • •

Scarring Ocular complications Recurrent infections Prolonged hospital stays

Aronson PL. Pediatrics. 2011. Aronson PL x 2. Pediatr Dermatol. 2013.

Summary

Thank You!

• Case 1: Stevens-Johnson Syndrome – Watch for atypical targets, classic mucous membrane involvement, calculate BSA

• Case 2: Staph Scalded Skin Syndrome – Look for a superficial epidermal split, non-toxic child, culture potential sources, can do a frozen section

• Case 3: Eczema Herpeticum – Look for monomorphous erosions in a patient with AD, consult ophtho if close to eyes, prompt acyclovir

[email protected]

References

References

(cont)

• Bastuji-Garin S, Razny B, Stern RS, Shear H, Naldi L, Roujeau J. Clinical classification of cases of toxic epidermal necrolysis, Stevens- Johnson syndrome, and erythema multiforme. Arch Dermatol 1993;129(1):92-6.

• Aronson PL, Yan AC, Mittal MK, Mohamad Z, Shah SS. Delayed acyclovir and outcomes of children hospitalized with eczema herpeticum. Pediatrics. 2011 Dec;128(6):1161-7. doi: 10.1542/peds.2011-0948. Epub 2011 Nov 14. PubMed PMID: 22084327; PubMed Central PMCID: PMC3387896.

• Metry DW, Jung P, Levy ML. Use of intravenous immunoglobulin in children with stevens-johnson syndrome and toxic epidermal necrolysis: seven cases and review of the literature. Pediatrics 2003 Dec;112(6 Pt 1):1430-6

• Aronson PL, Yan AC, Mohamad Z, Mittal MK, Shah SS. Empiric Antibiotics and Outcomes of Children Hospitalized with Eczema Herpeticum. Pediatr Dermatol. 2013 Mar;30(2):207-214. doi: 10.1111/j.1525-1470.2012.01860.x. Epub 2012 Sep 20. PubMed PMID: 22994962.

• Finkelstein Y, Soon GS, Acuna P, George M, Pope E, Ito S, Shear NH, Koren G, Shannon MW, Garcia-Bournissen F. Recurrence and Outcomes of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children. Pediatrics. 2011 Sep 2.

• Aronson PL, Shah SS, Mohamad Z, Yan AC. Topical Corticosteroids and Hospital Length of Stay in Children with Eczema Herpeticum. Pediatr Dermatol. 2013 Mar;30(2):215221. doi: 10.1111/j.1525-1470.2012.01859.x. Epub 2012 Oct 5. PubMed PMID: 23039248. • Macias ES, Pereira FA, Rietkerk W, Safai B. Superantigens in dermatology. J Am Acad Dermatol. 2011 Mar;64(3):455-72; quiz 473-4. Review.