Disclosures - New Jersey AAP – New Jersey Chapter...

Eichenfield: Neonatal Dermatology

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What You Need to Know about

Pediatric DermatologyLawrence 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 malformationsFrieden 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

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

696 molluscum patients: mean age, 5.5 yrs • Molluscum dermatitis: 39%

• Inflamed MC: 22%

• Gianotti-Crosti like: 5%

Atopic Dermatitis:

• Risk factor for MC

• More MC-dermatitis (51%)

Molluscum-Associated Gianotti-Crosti

Like Syndrome• Id-like: like with tinea or disseminated

eczema with allergic contact dermatitis

• 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

• DyspigmentationEichenfield 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):

BenefitsBenefits:• Highly effective • Rapid onset of action

• Multiple potency and delivery vehicles

Mainstay of therapy for Atopic Dermatitis

• Anti-inflammatory

• Used for acute flare management

• Intermittently for maintenance therapyEichenfield LF, et al. J Am Acad Dermatol. 2014;71:116-132. Stein SL, et al. JAMA. 2016.315:1510-1511.

Topical Corticosteroid Potency

Eichenfield LF et al.

Am Acad Dermatol.

2014 Jul;71(1):116-

32


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Pediatric Steroid Safety StudiesAuthor Drug Design # Pts Results

Crespi Aclometasone creamOpen BID

3wks

39Nl AM cortisols

LuckyDesonide 0.5%

HC 2.5%

Open, parallel

BID x 4 wks20 CST all WNL

Friedlander Fluticasone creamOpen MC,

Bid 3-4 weeks

51

BSA -64%

3mos 5yrs

2 abnl CST tests

Hanifin/SPMometasone

cream/HC cream

MC, R, , Par 3

wks

62;

6mos-2 yrs1 abnl CST -Elocon

MoshangPrednicarbate cream

0.1%

Open bid

3 wks

55;

4 mths-12 rsCST all WNL

PallerFluocinolone .01% in

oil

Open MC, BID x 4

wks

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2-12yrsCST all WNL

EichenfieldDesonide 0.05% gel

(DESONATE)Open bid x 4 wks

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3mos-6 yrsCST all WNL

Adapted Stepwise AD Management

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

Low to mid potency TCS

Low/Mid/High Potency TCS

PROACTIVE TCS/TCI

Low/Mid/HIGH Potency TCS +/-

Step 5

Step 3

Step 4

SYSTEMIC THERAPY: Trad or

Dupilimab-


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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.

http://www.ncbi.nlm.nih.gov/pubmed/21967469

http://www.ncbi.nlm.nih.gov/pubmed/21967469

Disclosures - New Jersey AAP – New Jersey Chapter...

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