M O I S E L . L E V Y , M . D . D E L L C H I L D R E N ’ S M E D I C A L C E N T E R D E L L M E D I C A L S C H O O L / U T , A U S T I N

A U S T I N , T X

A Potpourri of Pediatric Dermatology 4th Annual Texas Children’s Hospital

Advanced Practice Provider Conference April 2017

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

-14 y/o female -Chronic abd pain -Exploratory lap -Cholecystectomy -Recurrent painful erythema

Oops!!!

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

Exanthem and fever more severe Hospitalization more common… - dehydration, pain

MMWR 2012;61:213 Mathes, et al: SPD, July 2012 JAAD 2013;69:736 Pediatrics 2013;132:e149

Severe HFM and Coxsackie A6

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, observed in most cases of AD

Suggestive of AD, but too nonspecific to be used for defining or detecting AD in research or epidemiologic studies

1.  Pruritus 2.  Eczema (acute, subacute, chronic)

a.  Typical morphology and age- specific patterns •  Infants/children: facial, neck, and

exterior involvement •  Any age group: current or

previous, flexural lesions •  Sparing of the groin and axillary

regions b.  Chronic or relapsing history

1.  Early age of onset 2.  Atopy

a.  Personal or family history b.  Immunoglobulin E (IgE)

reactivity 3.  Xerosis

1.  Atypical vascular responses (eg, facial pallor, white dermographism, delayed blanch response)

2.  Keratosis pilaris/pityriasis alba/hyperlinear palms/ichthyosis

3.  Ocular/periorbital changes 4.  Other regional findings (eg, perioral

changes/periauricular lesions) 5.  Perifollicular accentuation,

lichenification, or prurigo lesions

Exclusionary Conditions

Diagnosis of AD depends on excluding conditions

•  Scabies •  Psoriasis •  Ichthyoses

•  Seborrheic dermatitis •  Contact dermatitis

(irritant or allergic) •  Cutaneous T-cell lymphoma

•  Photosensitivity dermatoses •  Immune deficiency diseases •  Erythroderma of other causes

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

23

Eichenfield LF et al. Pediatrics. 2015;136:554-565.

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

Topical anti-inflammatory medication applied to inflamed skin

Low-potency topical corticosteroids (class VII) twice daily for up to 3 days beyond clearance

Acute Treatment

“Flare” (acute worsening of symptoms, necessitating escalation in treatment)

1. Skin care a.  Moisturizera (choice depends on patient

preference) liberal and frequent b.  Warm baths or showers using nonsoap

cleansers or mild soaps generally once daily followed by application of moisturizera (even to “uninvolved” skin)

2. Antiseptic measures 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 Avoid common irritants (eg, soaps, wool), temperature extremes, and proven allergens

Basic Management

(pimecrolimus or tacrolimus)

TCI 2 to 3 times weekly19-22

OR (if patient is

nonresponsive) TCI once to twice daily22,24,25

Maintenance TCI

Medium-potency topical corticosteroids (class III-IV, see Table 3) once to twice

weekly (except for face/eyes)23

AND/OR (depending on patient/physician

preference and lesion location)

Low-potency topical corticosteroids (class V-VII, see Table 3) once to twice

daily (including face and eyes)

Maintenance Topical Corticosteroids

For relapsing course (frequent/persistent flares despite treatment) Topical anti-inflammatory medication applied at first signs/symptoms

or to flare-prone areas

Topical anti-inflammatory medication applied to inflamed skin

Medium-potency topical corticosteroids (class III-IV, see Table 3) twice daily for up to 3 days beyond clearance

Consider possible secondary infection that may require oral antibiotic

Acute Treatment

Flare not resolved within

7 days

Consider nonadherence,

infection, misdiagnosis,

referral

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 Restoraderm

10 oz $14.88

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-infectives

Anti-convulsants

Anti-hypertensives

Biologics NSAIDs Misc

Dapsone Carbemazepine

Amlodipine

Imatinib Celecoxib Allopurinol

Ampicillin Lamotrigine

Captopril Vismodegib

Ibuprofen Ranitidine

Linezolid PBS Vemurafenib

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

Then Adolescent Acne: 12-18?

AcneType AgeRangeNeonatal 0-6wk

Infan0le 0-1yr

Mid-childhood 1-7yr

Preadolescent 7-12yr

Adolescent 12-19yr

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 UnitEpiduo (.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

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?

Case History

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?

GREAT EXPERIENCE Convenient Close Familiar Friendly Caring Professional Respectful Attentive Prompt Thorough New Available Clean Nice Comfortable

GREAT CARE Quality Doctors Quality Care Quality Nurses Good Hospital Doctor Affiliation Maternity Pediatric Cardiac Specialists Surgical Equipment Orthopedic

25% 75%

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

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