Dra. Nicole Knöpfel CapelinhaHospital Universitario Son EspasesPalma de Mallorca

PEDIATRICS

Optimal Management of Pediatric MorpheaYvonne Chiu, MDCircumscribed (plaque); Linear (limb/trunk/head - En coup de sabre - Parry-Romberg syndrome); Generalized; Pansclerotic; Mixed

Pediatric MorpheaA severe disease: Linear the most common subtype in children and more likely to haveextracutaneous manifestations

Neurological 20-40%Musculoskeletal-articular 20-50%Ocular involvement 2-3%Other autoimmune disorders 2%

En coup de sabre:Poor correlation between MRI findings and neurological symptomsMayo experience: 48% with neurological symptoms - normal MRI vs 21% with abnormal MRI - no neurologicalsymptoms. Also poor correlation between MRI findings and clinical findings.

Neurological manifestations present after cutaneous disease in nearly all cases, but weaware of headache as a presenting sign: Importance of a dermatologist evaluationwhen changes are subtle.

Risk of extracutaneousmanifestations

Odds Ratio (95% CI)

p-value

Linear morphea 38% 22.3 (2.8-178) 0.00035

Circumscribed morphea 3%

Age of onset <10 y 36% 10.0 (2.1-47.6) 0.0036

Age of onset >10 y 5%Pequet el al. 2014

Optimal Management of Pediatric MorpheaYvonne Chiu, MDPediatric Morphea Work-Up: En Coup de Sabre or Parry-Romberg syndrome MRI at diagnosis (even if asymptomatic it may be useful for the future to have a baseline MRI) Repeat MRI if symptoms develop or worsen EEG at diagnosis if seizures are suspected Ophthalmology exam if eye complications are suspected

Linea morphea on limb Joint exam at each visit Physical therapy if functional limitations Leg length x-ray if limb length discrepancy and if necessary Trauma surgeon consultation

Pediatric Morphea Monitoring and Treatment: Clinical examination is the mainstay of disease activity assessment. Serological screening is not indicated or helpful. Positive findings are not indicative of disease activity. Treatment Ladder: Topical therapy for localized dermal lesions; Phototherapy for widespread dermal lesions (NBUVB and UVA1) Systemic Therapy: subcutaneous, muscle and bone involvement, linear morphea, face.

How does she do it: First line choice: Metilprednisolone 30mg/kg/dose IV 3 consecutive daily doses a month for 3 months +

Methotrexate 1mg/kg/week sc (max 25mg). Folic acid 1mg daily. Second line choice: Mycophenolate mofetil (600-1200mg/m2/day)

Improving Outcomes with Group Visits for Pediatric Atopic DermatitisMargaret S. Lee, MD PhDAD is a chronic disease which must be managed rather than cured

Strategies to enhance adherence and improve outcome Written eczema action plan Nurse-led eczema workshops (30min)

Atopic dermatitis educator (15min)

Schedule follow-up visit closer to the original appointment “White coat adherence”

Parental training program Educational program: group visits GOALS: SELF-MANAGEMENT CARE; PSYCHOLOGICAL SUPPORT; MOTIVATION; Small groups Age-appropriate Share relaxation techniques – “instant itch relief” interventions

The best type of education program is not known.. and most certainly will depend on the work-setting, professional experience among others.

Highlights From the Pediatric LiteratureHoward B. Pride, MD Nevus anemicus and juvenile xanthogranuloma could be major NF1 criteria

“Nevus anemicus: a distinctive cutaneous finding in neurofibromatosis type 1” Hernández-Martin et al. Pediatr Dermatol 2015 Mycoplasma-associated SJS is probably a different beast than SSJ/EM.

MIRM has a distinct morphology, mild disease course, and potentially important clinicalimplications“Mycoplasma pneumoniae-induced rash and mucositis as a syndrome distinct from Stevens-Johnson syndrome and erythema multiform: a

systematic review” Canavan et al. JAAD 2015

Peanut Allergy is decreased by early exposure. Now what?“Randomized trial of peanut consumption in infants at risk for peanut allergy” Du Toit et al. NEJM 2015

The genetics of port wine stains and Sturge-Weber syndrome have been discovered.We can stop talking about dermatomes.“Sturge–Weber Syndrome and Port-Wine Stains Caused by Somatic Mutation in GNAQ” Shirley et al. NEJM 2013“The somatic GNAQ mutation c.548G>A (p.R183Q) is consistently found in Sturge-Weber syndrome.” Nakashima et al. J Hum Genet 2014

Sirolimus: The next Propanolol?“Efficacy and Safety of Sirolimus in the Treatment of Complicated Vascular Anomalies” Adams et al. Pediatrics 2016“Sirolimus for the treatment of children with various complicated vascular anomalies” Lackner et al. Eur J Pediatr 2015

“Infantile Hemangiomas in Twins: A Prospective Cohort Study”Fernanda Greco F, Frieden I, Drolet B et al. Pediatr Dermatol 2016

“Beyond Psoriasis: Novel Uses for Biologic Response Modifiers in Pediatric Dermatology”F Bellodi-Schmidt F and Shah K Pediatr Dermatol 2016

A Practical Approach to Pediatric Autoimmune Connective Tissue DiseasesAlisa Femia, MDSession focused on single case presentations – Adult and Pediatric formsLupus Erythematosus in the pediatric population Neonatal Lupus Erythematosus

Due to transplacental passage of maternal anti-Ro antibodiesSkin involvement most frequent manifestationCardiac involvement in 65% (82% detected in utero)Hepatobiliary, hematologic involvement

Discoid Lupus ErythematosusLess common in children than in adultsEqual sex distribution prior to pubertyHigher progression to Systemic LE than initially suspected

Juvenile Dermatomyositis (JDM)EARLY and AGGRESSIVE management Better Outcome! Methotrexate to reduce sequelae IVIG particularly helpful for refractory cases

Greater interest in long-term outcomes: physical function, pain, quality of life,educational achievement and ongoing disease activity