Eichenfield: Neonatal Dermatology

What You Need to Know about Pediatric Dermatology Lawrence F. Eichenfield, M.D. Professor of Dermatology and Pediatrics University of California, San Diego Rady Children’s Hospital, San Diego

Disclosures No conflict of interest for this talk

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Demographics / Risk • 3-10% of infants (up to 30% in extremely premature babies) – San Diego, US data = 4.5% – Complications in 12-27% (ulceration, impaired vision, disfigurement) • M:F = 1:1-4 – Females have more severe IH • Risk factors: 5 – Caucasian – Female – Prematurity – Placental anomalies

The most appropriate workup is: A. MRI of the brain B. Echocardiogram C. Ultrasound and/or MRI of great vessels D. Ophthalmology examination E. All of the above F. Reassurance (and none of the above)

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PHACE Syndrome (OMIM 606519) • Posterior fossa brain abnormalities • Hemangiomas • Arterial malformations • Coarctation of the aorta and cardiac defects • Eye abnormalities • PHACES syndrome- includes sternal malformations Frieden IJ et al. 1996. Arch Dermatol 132:309-311.

PHACE SYNDROME: DIAGNOSTIC CRITERIA • Facial Hemangioma 5 cm in diameter PLUS • 1 Major Criteria OR 2 Minor Criteria

Metry D, Heyer G et al. Pediatrics. 2009 Nov;124(5):1447-56.

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Propranolol Propranolol hydrochloride oral solution • FDA-approved prescription product available –4.28 mg/mL propranolol hydrochloride –Dosing 2.2 mg/kg-3.3 mg/kg •Generic dosing 2-3 mg/kg

Propranolol: Hemangiomas • FDA-approved medication • RDB Phase 2-3 trial: 1 or 3 mg/kg 3 or 6 mths • After first 188 patients completed 24 weeks 3 mg/kg per day for 6 months • Successful treatment 60% vs. Placebo: 4%, P<0.001 • 88% showed improvement by wk 5, versus 5% of placebo

Léauté-Labrèze C, Hoeger P et al. N Engl J Med. 2015;Feb 19;372(8):735-46

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Topical Beta-Blockers • Topical Timolol (gel-forming solution) –Used topically for early, flat lesions –Medication OFF-LABEL, more potent mg per mg than propranolol

Chakkittakandiyil A et al. Pediatr Dermatol. 2012;29(1):28-31 McMahon P et al. Pediatr Dermatol. 2012;29(1):127-30 Moehrle M, Léauté-Labrèze C. Pediatr Dermatol. 2013 Mar-Apr;30(2):245-9

Molluscum Contagiosum:

• DNA Pox virus – Virus has been sequenced – Not yet propagated in culture • Cutaneous infection only • Contact spread • Truly are self-limited….BUT…

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MOLLUSCUM- The Bump That Rashes: Inflammatory Reactions 696 molluscum patients: mean age, 5.5 yrs • Molluscum : 39% • Inflamed MC: 22% • Gianotti-Crosti like: 5% : • Risk factor for MC • More MC-dermatitis (51%)

Berger E et al. Arch Dermatol 2012 Nov;148(11):1257-64

Molluscum-Associated Gianotti-Crosti Like Syndrome • Id-like: like with tinea or disseminated eczema with allergic • More pruritic • More localized to elbows and knees • Better response to topical corticosteroids

Berger E et al. Arch Dermatol 2012 Nov;148(11):1257-64

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Name the Infection • Transmission to other children: 41% of cases • 30% of cases persist past 18 months – (13% 24 mths) • 11% have marked QOL decrease Mean resolution: 13.3 mths Olsen JR et al. Lancet Infect Dis 2015;15:190-5

Differential Diagnosis of Atopic Dermatitis: Common Disorders • Seborrheic dermatitis • Scabies • Contact dermatitis (allergic and irritant) • Psoriasis • Ichthyosis vulgaris • Tinea corporis • Keratosis pilaris

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Characteristic Findings: Atopic Dermatitis

• Erythema • Papules/vesicles • Excoriations • Xerosis • Erosions • Lichenification • Dyspigmentation Eichenfield LF, et al. Pediatrics. 2015;136(3):554-565; .Siegfried EC, et al. J Clin Med. 2015;4(5):884-917.

Therapy Basics

• Bathing • Moisturizers: After bathing plus… • Anti-inflammatory Rx – Top Corticosteroids – Second Line: Top Calcineurin Inhibitors – Topical PDE-4

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Topical Corticosteroids (TCS): Benefits Benefits: • Highly effective • Rapid onset of action • Multiple potency and delivery vehicles

Mainstay of therapy for Atopic Dermatitis • Anti-inflammatory • Used for acute flare management Eichenfield LF, et al. J Am Acad Dermatol. 2014;71:116-132. Stein SL, et al. JAMA. 2016.315:1510-1511. • Intermittently for maintenance therapy

Topical Corticosteroid Potency

Eichenfield LF et al. Am Acad Dermatol. 2014 Jul;71(1):116- 32

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Pediatric Steroid Safety Studies Author Drug Design # Pts Results Open BID 39 Crespi Aclometasone cream Nl AM cortisols 3wks Desonide 0.5% Open, parallel Lucky 20 CST all WNL HC 2.5% BID x 4 wks 51 Open MC, Friedlander Fluticasone cream BSA -64% 2 abnl CST tests Bid 3-4 weeks 3mos 5yrs Mometasone MC, R, , Par 3 62; Hanifin/SP 1 abnl CST -Elocon cream/HC cream wks 6mos-2 yrs Prednicarbate cream Open bid 55; Moshang CST all WNL 0.1% 3 wks 4 mths-12 rs Fluocinolone .01% in Open MC, BID x 4 32 Paller CST all WNL oil wks 2-12yrs

Desonide 0.05% gel 34 Eichenfield Open bid x 4 wks CST all WNL (DESONATE) 3mos-6 yrs

Adapted Stepwise AD Management

SYSTEMIC THERAPY: Trad or Step 5 Dupilimab-

PROACTIVE TCS/TCI Step 4 Low/Mid/HIGH Potency TCS +/-

Step 3 Low/Mid/High Potency TCS

Low to mid potency TCS

Adapted from Akdis CA, et al. J Allergy Clin Immunol 2006;118:152

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Staph Scalded Skin Syndrome • Seen in: neonates, young children; can be seen in older children to adulthood • Early symptoms: Fever, malaise, irritability, followed by erythema and patches diffusely with fragile bullae, peeling, denuded “scalded appearance” – Common:Accentuation in folds (neck, axillae, perineal areas); “Sunburst pattern” by mouth

Staph Scalded Skin Syndrome • Exfoliating (epidermolytic) toxin (ET) from phage II Staphylococcus aureus • 2 types of ETs: ETA and ETB (mostly A in West) – Target: desmoglein-1, a cell-to-cell adhesion molecule in the stratum granulosum

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SSSS: Differential Dx • Toxic epidermal necrolysis (TEN) • Drug reaction with eosinophilia and systemic symptoms (DRESS) • Staphylococcal Toxic-Shock Syndrome (STSS) • Allergic contact Dermatitis • Enteroviral Infection

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Terra firma-forme dermatosis • Dirt-like plaques develop on the skin despite normal hygiene • Terra firma means “solid earth” in Latin • Brown or black hyperkeratotic plaques • Berk et al: 31 cases: Neck, ankles, face most commonly affected areas Berk DR. Pediatr Dermatol. 201;29(3):297-300.

Differential Dx • Acanthosis nigricans • Confluent and reticulated papillomatosis (CARP) • Pityriasis versicolor

Berk DR. Pediatr Dermatol. 2012 May-Jun;29(3):297-300.

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