A Potpourri of Pediatric Dermatology 4th Annual Texas Children’s Hospital Advanced Practice Provider Conference April 2017

MOISE L. LEVY, M.D. DELL CHILDREN’S MEDICAL CENTER DELL MEDICAL SCHOOL/UT, AUSTIN AUSTIN, TX Conflicts of Interest

Ø Anacor Ø Scioderm Ø Castle Creek Pharmaceuticals Ø Up to Date

None Should Apply For This Presentation One way we are viewed… It’s just a birthmark

9 month old with asymptomatic scalp lesion noted in NICU; increases in size with straining But maybe something else… Sinus Pericranii

Ø Communication between intra- and extracranial venous drainage pathways Ø Most are midline and non.pulsatile Ø Connect pericranial veins with superior sagittal sinus Ø TX depends on flow pattern/direction -Dominant; main flow via SP -Accessory; small flow via SP

Neuroradiology 2007;49:505 Neurology 2009;72:e66 Case History

— 5 y/o girl w celiac disease — Seen for evaluation of perianal growth — Present, by hx, x 2 yrs — No prior tx

Case History

— Was seen by pediatric surgery — Excised — Condyloma - HPV 6, 16 — Management? ARS – Pediatric Anogenital Warts

— When seeing a 5 yo with anogenital warts

A. HPV testing should be done B. All cases are due to abuse C. All cases should be treated with imiquimod D. History and physical examination are key for suspicion of abuse E. Call Dr Eichenfield; he’ll know what to do Pediatric Anogenital Warts

— Age of onset and abuse

- 6.5 ± 3.8 yrs (5.3 yrs)

- 37% 2-12 yrs; 70% > 8 yrs

- 4 yrs 8 months (83% female) — HPV testing felt not of use… high subclinical — History and PE key

- physical findings abuse rare

Arch Dermatol 1990;126:1575 Pediatrics 2005;116:815 Arch Dis Child 2006;91:696 Pediatr Dermatol 2006;23:199 J Pediatr Adolesc Gynecol 2013;26:e121

Age of Onset and Abuse

Pediatr Dermatol 2006;23:199 ARS – Pediatric Anogenital Warts

— When seeing a 5 yo with anogenital warts

A. HPV testing should be done B. All cases are due to abuse C. All cases should be treated with imiquimod D. History and physical examination are key for suspicion of abuse E. Call Drs Hunt or Metry; they’ll know what to do Oops!!!

-14 y/o female

-Chronic abd pain

-Exploratory lap

-Cholecystectomy

-Recurrent painful erythema Case History/Factitious n Dermatographia n Photographed n Severe behavioral disorder n Psychotherapy Lessons Learned

— Listen to the trainees…

— Reason for being in teaching environments is to be challenged by everyone Hand-Foot-and-Mouth Disease Hand Foot and Mouth

— Sausage shaped vesicles palms/soles — Oral erosions - Can see feeding problems — Erythematous papules on buttocks — “Eczema coxsackium” — Mild gastroenteritis — Etiologies -Coxsackie A16, A2, A5, A6, A9, A10, B2… -Enterovirus 71 -Others…

Pediatrics 2013;132:e149 Severe HFM and Coxsackie A6

MMWR 2012;61:213 Exanthem and fever more Mathes, et al: SPD, July 2012 severe JAAD 2013;69:736 Hospitalization more Pediatrics 2013;132:e149 common… - dehydration, pain Atopic Dermatitis: The Disease

— Increasing prevalence1 ¡ 15%-30% of general population ¡ 80%-90% diagnosed by 5 years of age — Pathogenesis1-3 ¡ Genetic; filaggrin other biomarkers ¡ Increased transepidermal water loss; possible lipids and barrier? ¡ Biochemical immunologic ÷ Potential food hypersensitivity? ÷ Hygiene hypothesis? ÷ Defective innate immunity — Management

Atopic Dermatitis: AAD Diagnostic Criteria1,2 20

Essential Features Important Features Associated Features

Both of the following must be present Add support to the diagnosis, Suggestive of AD, but too nonspecific observed in most cases of AD to be used for defining or detecting AD in research or epidemiologic studies 1. Pruritus 1. Early age of onset 1. Atypical vascular responses (eg, 2. Eczema (acute, subacute, chronic) 2. Atopy facial pallor, white dermographism, delayed blanch response) a. Typical morphology and age- a. Personal or family history 2. Keratosis pilaris/pityriasis alba/ specific patterns b. Immunoglobulin E (IgE) hyperlinear palms/ichthyosis • Infants/children: facial, neck, and reactivity exterior involvement 3. Xerosis 3. Ocular/periorbital changes • Any age group: current or 4. Other regional findings (eg, perioral previous, flexural lesions changes/periauricular lesions) • Sparing of the groin and axillary 5. Perifollicular accentuation, regions lichenification, or prurigo lesions b. Chronic or relapsing history

Exclusionary Conditions

Diagnosis of AD depends on excluding conditions • Scabies • Seborrheic dermatitis • Photosensitivity dermatoses • Psoriasis • Contact dermatitis • Immune deficiency diseases • Ichthyoses (irritant or allergic) • Erythroderma of other causes • Cutaneous T-cell lymphoma

J Am Acad Dermatol 2014;70:338 Atopic Dermatitis: Distribution 21

Weidinger S et al. Lancet. Published online ahead of print September 14, 2015. Lancet 2016;387:1109 “Eczema Coxsackium”

Pediatrics 2013;132:e149 Atopic Dermatitis: Treatment Guidelines

Mild Disease 23 Moderate to Severe Disease Basic Management for All patients at All times (add maintenance and/or acute treatment as needed)

Basic Management Maintenance TCI Maintenance Topical

Corticosteroids 1. Skin care (pimecrolimus or tacrolimus) Medium-potency topical corticosteroids a. Moisturizera (choice depends on patient (class III-IV, see Table 3) once to twice 19-22 preference) liberal and frequent TCI 2 to 3 times weekly weekly (except for face/eyes)23 OR b. Warm baths or showers using nonsoap (if patient is AND/OR (depending on patient/physician cleansers or mild soaps generally once daily nonresponsive) preference and lesion location) 22,24,25 followed by application of moisturizera (even to TCI once to twice daily “uninvolved” skin) Low-potency topical corticosteroids (class V-VII, see Table 3) once to twice 2. Antiseptic measures daily (including face and eyes) Diluted bleach bathsb (or equivalent) twice weekly or more (daily for more severely affected children), especially for patients with recurrent skin infections 3. Trigger avoidance For relapsing course (frequent/persistent flares despite treatment) Avoid common irritants (eg, soaps, wool), Topical anti-inflammatory medication applied at first signs/symptoms temperature extremes, and proven allergens or to flare-prone areas

Flare not resolved within 7 days

Acute Treatment Acute Treatment Topical anti-inflammatory medication Consider Topical anti-inflammatory medication applied to inflamed skin nonadherence, applied to inflamed skin Medium-potency topical corticosteroids (class III-IV, infection, Low-potency topical corticosteroids (class VII) twice daily see Table 3) twice daily for up to 3 days beyond clearance misdiagnosis, for up to 3 days beyond clearance Consider possible secondary infection that may referral require oral antibiotic “Flare” (acute worsening of symptoms, necessitating escalation in treatment)

Eichenfield LF et al. Pediatrics. 2015;136:554-565. Psychology Referral… early, as needed! Moisturizer Size Cost (Avg)

Vaseline 13 oz $4.51

Eucerin Cream 16 oz $12.88

Cetaphil Lotion 16 oz $10.45

Cetaphil 10 oz $14.88 Restoraderm

Aquaphor 14 oz $15.44

Aveeno Ecz Care 7.3 oz $12.42

Cerave Cream 16 oz $15.10

Vanicream 16 oz $13.19

Epiceram 90 g $175.30 Mycoplasma pneumoniae-Induced Rash and Mucositis (MIRM)

— Difference from drug or viral induced SJS/EM — Generally w more limited skin involvement - vesiculobullous

— Oral>Ocular>GU mucosal involvement - mucositis alone seen in 34% — Most with excellent recovery; ATB (!), IVIG, steroids - resistance (TCN or fluoroquinolone) — Recurrence rarely

J Am Acad Dermatol 2015;72: 239 Pediatrics 2011;127:e1605

Photos from: J Am Acad Dermatol 2105;72:239 Case History

Ø A child is seen with rapidly developing morbilliform erythema, facial edema, and fever. These findings were noted approximately 21 days after starting carbamazepine. Laboratory studies show 13% eosinophilia and elevated liver functions. Case

— 1. Stevens-Johnson syndrome — 2. Serum sickness – like eruption — 3. Urticaria multiforme/Giant urticaria — 4. Drug reaction with eosinophilia and systemic symptoms (DRESS) — 5. Presidential fever Case

— 1. Stevens-Johnson syndrome — 2. Serum sickness – like eruption — 3. Urticaria multiforme/Giant urticaria — 4. Drug reaction with eosinophilia and systemic symptoms (DRESS) — 5. Presidential fever Case

Which of the following viral conditions is associated with DRESS? A – Echovirus 8 infection B – Hepatitis A infection C – HHV-6 reactivation D – Influenza A E – Parvovirus infection Case Case

Ø A – Echovirus 8 infection; has not been reported Ø B – Hepatitis A infection; has not been reported Ø C – HHV-6 reactivation; multiple reports of such; ? Due to cytotoxic T cell activation w cross reaction with drug and organ toxicity (also EBV, CMV) Ø D – Influenza A; has not been reported Ø E – Parvovirus infection; has not been reported DRESS – Associated Medications

Anti- Anti- Anti- Biologics NSAIDs Misc infectives convulsa hypertens nts ives Dapsone Carbemazepin Amlodipin Imatinib Celecoxib Allopurino e e l Ampicillin Lamotrigin Captopril Vismodegi Ibuprofen Ranitidine e b Linezolid PBS Vemurafen ib Minocycline Phenytoin TMP/SMX Valproate Vancomycin INH DRESS – Systemic Concerns

— Autoimmune disease – - Grave’s - Type 1 DM - AA - Autoimmune hemolytic anemia

— End organ disease… most commonly renal

Semin Cutan Med Surg 2014;33:2 DRESS - Management

— Stop the suspected medication — Steroids… systemic v topical — Retrospective study - 50 consecutive pts with d/c Dx: 38 prob or confirmed — Potent topical steroids v Systemic steroids - Systemic tx not felt nec for mild disease - Systemic use felt to be assoc with viral reactivation (HHV6) and more severe/prolonged course (*)

JAAD 2015;72:246 DRESS and HHV 6 Corticosteroids

— Retrospective evaluation of 29 pediatric cases — All HHV6 + and 80% of HHV6 – pts received CS — Shorter time to cessation of progression of disease — Fewer days with fever — Suggestion of shorter hospitalization — Conclusion of more severe illness with HHV6 + and better outcome in both with systemic steroid use

Br J Dermatol. 2015 Apr;172(4):1090-5

Acne – Clinical Description

— Comedone — Papule — Pustule — Nodule/Cyst — Scarring Acne Grading and Classification

Ø Simplicity is the key Ø Associating descriptions of lesions noted with extent of involvement to arrive at final assessment of severity Ø Must include assessment of scarring and pigmentation Ø Must include assessment of impact on individual Acne Severity and Classification Therapeutic Implications

Ø Let the lesion dictate the therapy

-Take clinical assessment as baseline evaluation -Consider scarring, pigmentation Ø Engage the patient in the process

-What is their assessment of severity? -Will they participate in the therapy? Ø We favor description of disease and assessment of overall severity to guide ultimate therapy Acne Categorization by Age

Acne Type Age Range Neonatal 0-6 wk

Infanle 0-1 yr

Mid-childhood Then Adolescent1- 7 Acne:yr Preadolescent 12-18? 7-12 yr

Adolescent 12-19 yr Evaluation/work-up: By Age Groups

— Neonatal

— Infantile

— Mid-Childhood – most likely time to have underlying endocrine abnormality

— Pre-Adolescent Acne

— Adolescent Acne Acne - Management

— Local skin care — Retinoid; tretinoin, adapalene, tazarotene — Benzoyl peroxide — Topical antibiotic; clindamycin, sodium sulfacetamide… — Combination pdts; — Oral antibiotics — Isotretinoin; I Pledge

Pediatrics 2013;131:s163 JAAD 2016;74:945 Acne Treatment Algorithm

J Am Acad Dermatol 2016;74:945 Drug Price Price Per Unit Epiduo (.01%-2.5%) Qty: 45grams $ 284.00 $ 6.32 Clindamycin/Benzoyl Peroxide Gel • 5%-1% 25 grams $ 159.14 $ 6.37 • 5%-1.2 45 grams $ 158.41 $ 3.52 Tretinoin Cream .05 • Qty: 20 grams $ 56.19 $ 2.81 • Qty: 45 grams $ 118.92 $ 2.64 Adapalene Qty: 45 grams $ 182.90 $ 4.06 Doxycycline Monohydrate 50 mg Qty: 20 $ 26.92 $ 1.35 Doxycycline Monohydrate 100mg Qty: 20 $ 30.75 $ 1.54 Doxy Hyclate 50 Qty: 20 $ 15.20 $ 0.76 Doxy Hyclate 100 Qty: 20 $ 14.08 $ 0.70 Minocycline 50 Qty: 30 $ 31.43 $ 1.05 Minocycline 100 Qty: 30 $ 43.68 $ 1.46 Doxepin Gel - 10mg Qty: 30 $ 15.18 $ 0.51 Trimethoprim/Sulfamethoxazole DS 200mg-40mg/5ml Qty: 100ml $ 21.26 $ 0.21 Acne – When to Refer

— When comedonal/inflammatory disease is unresponsive after 3 months - topicals - orals — Endocrine evaluation; consider in pts 1-7 yrs

— If considering isotretinoin and before scarring; nodular/cystic or severe papulopustular

— Referral guidelines

Case Case

Increase in Incidence of Congenital Syphilis — United States, 2012–2014 November 13, 2015 / 64(44); 1241-1245 Case History

Ø A young girl comes in for evaluation of a pruritic eruption on her chin. She was recently at a swimming party. Ø What is your dx? Ø What is the cause? Phytophotodermatitis

Ø Phototoxic reaction between sunlight and plant product (furocoumarin/psoralens) Ø Hours after exposure; edema, erythema, occl. Vesiculation Ø Hyperpigmentation Ø Bizarre, geometric orientation Ø Lime, lemon, plantains, others!!! Phytophotodermatitis

Ø Onset usually w/in 1 day of contact

Ø Avoidance

Ø Compresses, if severe

Ø Topical steroids of possible use

Ø Hyperpigmentation persists months-years Transformative Teams in Healthcare Communication and Collaboration Seminar

— Students in: Medicine, Nursing, Social Work, Pharmacy, Educational Psychology

— Parents help facilitate discussion -Experience with dx -What has gone well… what went badly -What could be better -Did the “team” function as a team? -What were key things having greatest impact?

Survey results of why people prefer one healthcare organization over the other

GREAT EXPERIENCE GREAT CARE

Convenient Quality Doctors Close Quality Care Familiar Quality Nurses Friendly Good Hospital 25% 75% Caring Doctor Affiliation Professional Maternity Respectful Pediatric Attentive Cardiac Prompt Specialists Thorough Surgical New Equipment Available Orthopedic Clean Nice Comfortable Primary Care Model of Care

— Patient aligned care team (PACT) — Providers, Nurses, Assistants, Office administrators — Relationships built around patient needs — True collaboration with all team members — Coordination… not functioning in “traditional” role as dermatologist Harrod, et al. J Interprof Care 2016 “IF YOU WANT TO ENSURE YOUR EXTINCTION, CEASE TO EVOLVE”

(Jeff Bezos) Hopes for the new Medical Model

— Organization around medical conditions… not administrative needs — Measures and results on providers and treatments — Organize information/pt support around cycle of care — Comprehensive disease management and prevention to all

Porter and Teisberg. Redefining Health Care. Harvard Business School Publishing, 2006. To close

— “… One of the essential qualities of the clinician is interest in humanity, for the secret of the care of the patient is in caring for the patient.” Peabody FW. JAMA 1927