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 1
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) 2
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. 3
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 4
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 5
MOLLUSCUM- The Bump That Rashes: Inflammatory Reactions 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%) 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 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 6
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 7
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 8
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 9
Pediatric Steroid Safety Studies Author Drug Design # Pts Results Crespi Aclometasone cream Lucky Friedlander Hanifin/SP Moshang Paller Desonide 0.5% HC 2.5% Fluticasone cream Mometasone cream/hc cream Prednicarbate cream 0.1% Fluocinolone.01% in oil Open BID 3wks Open, parallel BID x 4 wks Open MC, Bid 3-4 weeks MC, R,, Par 3 wks Open bid 3 wks Open MC, BID x 4 wks 39 Nl AM cortisols 20 CST all WNL 51 BSA -64% 3mos 5yrs 62; 6mos-2 yrs 55; 4 mths-12 rs 32 2-12yrs 2 abnl CST tests 1 abnl CST -Elocon CST all WNL CST all WNL Eichenfield Desonide 0.05% gel (DESONATE) Open bid x 4 wks 34 3mos-6 yrs CST all WNL Adapted Stepwise AD Management Step 3 Step 4 Step 5 SYSTEMIC THERAPY: Trad or Dupilimab- PROACTIVE TCS/TCI Low/Mid/HIGH Potency TCS +/- Low/Mid/High Potency TCS Low to mid potency TCS Adapted from Akdis CA, et al. J Allergy Clin Immunol 2006;118:152 10
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, 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 11
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 12
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. 13