Objectives Identify, appropriately work-up, and Dermatologic - PDF document

5/9/2013 Objectives • Identify, appropriately work-up, and Dermatologic Emergencies: stabilize urgent/emergent dermatologic What’s That? conditions • 3 cases that illustrate: – Erythema Multiforme Erin Mathes, MD – Stevens-Johnson Syndrome Assistant Professor – Toxic Epidermal Necrolysis – Staph Scalded Skin Syndrome Dermatology and Pediatrics – Eczema herpeticum – Eczema coxsackium UCSF – Staph superinfection I have no relevant conflicts of interest. How to describe what you are Clues: When to Worry seeing... over the phone • A picture is worth a thousand words • Age (newborn and young infants) • High fever, toxicity • Extent: What body surface area is involved? • Morphology – the patient’s palm = 1% – blistering, mucosal involvement, hemorrhage • Specific medications – anti-convulsants, antibiotics, NSAIDS Case 1: Case 1: 5 yo with sz d/o and rash. What is the most likely diagnosis? 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 1. Erythema multiforme days, and lamotrigine for 6 weeks. 2. Kawasaki Disease 3. Stevens-Johnson syndrome (SJS) What is the most likely diagnosis? 4. Vasculitis 1

5/9/2013 Case 1 What is SJS? SJS vs EM vs TEN • Severe, life-threatening mucocutaneous disease • Clinical syndrome - no definitive diagnostic test • atypical “targetoid” lesions, fragility, denudation ~10%BSA • ≥ 2 mucous membranes (mouth, eyes) • systemic signs: fever, respiratory symptoms The SJS Spectrum The SJS Spectrum Erythema Multiforme Stevens Johnson Erythema Multiforme Stevens Johnson Minor and Major (SJS) Minor and Major (SJS) SJS-TEN SJS-TEN overlap overlap < 10% BSA < 10% BSA Toxic Epidermal Toxic Epidermal Necrolysis Necrolysis (TEN) (TEN) 10-30% BSA 10-30% BSA > 30% BSA > 30% BSA Infection Drug Low Mortality High mortality EM vs SJS vs TEN EM EM major SJS SJS-TEN TEN Rash Typical targets Typical targets Dusky red, Dusky red, Poorly delineated atypical targets, atypical targets, dusky plaques, Detachment Detachment large sheets of detachment BSA <10% 10-30% >30% with spots Detached >10% without spots Distribution Extremities, Extremities, Trunk, face Trunk, face Face, trunk, ext face face (+confluence) (++confluence) (+++confluence) Mucosal None, mild Severe Severe Severe Yes* Involvement Systemic Absent Usually Usually Always Always Symptoms Progression to No No Possible TEN Etiology HSV, other HSV, Drug Drug Drug infectious mycoplasma, Mycoplasma Bolognia. Dermatology . 2nd Edition. rare drug HSV 2

5/9/2013 Stevens-Johnson Syndrome (SJS) (Mycoplasma Associated) SJS: Causes • 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 Erythema Multiforme in EM Bolgnia, Dermatology , 2nd ed. 3

5/9/2013 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 SJS Initial Management TEN & Work-Up = Full thickness skin necrosis • ABCs • Stop the causative drug (and all non-essential drugs) Shiny dermis • Admit to ICU or burn unit if >10-20% BSA underneath • Call dermatology/ophthalmology/urology • Labs: CBC, Lytes, BUN, Cr, LFTs • Check for Mycoplasma, HSV (IgM) 4

5/9/2013 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 •Treat infection •Stop drug •Stop drug – Pressure bed •Steroids? •IVIG •Treat Infection – Avoid friction, trauma – Reverse isolation •Early steroids •IVIG? • Surveillance cultures (?) • Hydration (careful not to overload) • Nutrition (NG) Outcome of SJS/TEN spectrum What is going to happen to this child? 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 5

5/9/2013 Case 2: 8 yo with blistering. Diagnosis? Case 2 1. Kawasaki Disease 2. Staph Scalded Skin Syndrome 3. Toxic Epidermal Necrolysis SSSS vs TEN vs TSS 4. Toxic Shock Syndrome SSSS SSSS: Etiology • Begins as a localized, • Staph produces an exfoliative exotoxin often occult infection – Nasopharynx • Exotoxin cleaves desmoglein 1  – Perioral superficial epidermal cleft, acantholysis – Conjunctiva – Umbilicus – Paronychia – Wound – Urine – Middle Ear • Progresses to generalized erythema and skin fragility SSSS Staphyloccal Scalded Skin Syndrome • Clinical Presentation – Neonates: Widespread erythema, superficial erosions – Toddlers & Children: erythema, periorificial Source: blistering dactylitis scale and erosions, skin fragility and pain & conjunctivitis – Adults: rare - protective antitoxin Perioral furrowing, scale Skin pain 6

5/9/2013 Why isn’t this TEN? Why isn’t this TEN? Shiny = TEN Subepidermal split Not shiny = SSSS Superficial epidermal split Why isn’t this toxic shock syndrome? Toxic Shock Syndrome • Rarely a primarily cutaneous disease • Staph produces superantigens that cause: – fever – rash – hypotension – organ system involvement SSSS: Management Case 3: • Admit (especially in younger pts) A 13 yo girl with a history of atopic • Dermatology consult dermatitis presents with 1 day of a new rash around her eyes and mouth, and low • Culture potential sources grade fever. • Empiric anti-staph antibiotics (cover for MRSA) +/- Clindamycin – Clindamycin inhibits toxin production What is the best diagnosis? – d/c with abx based on cx results • Careful FEN management • Pain management 7

5/9/2013 Case 3: Best Diagnosis? Eczema Herpeticum 1. Contact Dermatitis 2. Eczema coxsackium • Disseminated HSV in 3. Eczema herpeticum pts with chronic skin dz 4. Staph superinfection • 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. Staph Superinfection Eczema Herpeticum vs. Contact Dermatitis 8

5/9/2013 Eczema Herpeticum vs. Eczema Coxsackium Strep Superinfection Eczema Herpeticum Eczema Herpeticum Treatment Sequelae • Culture, DFA or PCR • Scarring • Culture for bacteria • Ocular complications • Ophthalmology consult (for periocular involvement) • Recurrent infections • Dermatology consult • Prolonged hospital stays • Prompt high dose acyclovir • Empiric antibiotics if signs of bloodstream infection • Topical steroids okay • Avoid systemic steroids 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 mathese@derm.ucsf.edu • 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 9