Megha M. Tollefson, MD Associate Professor of Dermatology and Pediatrics, Mayo Clinic Pediatric Morphea March 1, MFMER

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1 Megha M. Tollefson, MD Associate Professor of Dermatology and Pediatrics, Mayo Clinic Pediatric Morphea March 1, MFMER

2 Disclosures None 2015 MFMER

3 Topics to cover Classification and Diagnosis Beyond the skin Treatment Things to consider when treating 2015 MFMER

4 Morphea Circumscribed Linear Generalized Pansclerotic Mixed Superficial Deep Head/neck Trunk/limbs 2015 MFMER

5 Genetics Congenital forms exist Fam hx of autoimmune dz Patterns of involvement? Immune factors Morphea Environmental factors Trauma Vascular injury 2015 MFMER

6 Making the Diagnosis Symptoms 1 year Diagnosis Laboratory findings Findings nonspecific Biopsy Clinical exam Red violaceous rim Warmth Raised borders Dermal thickening Morphea Imaging Kreuter et al JAAD MFMER

7 Imaging in Morphea Zulian et al. Curr Opin Rheumatol 2013, 25: MFMER

8 Beware of Mimics Weibel et al BJD 2011; 2015 MFMER

9 Call et al Ped Dermatol MFMER

10 Morphea stages 2015 MFMER

11 2015 MFMER

12 How can you tell that it s active? LoSSI LoSDI LoSCAT DIET Computerized skin score Infrared thermography Doppler ultrasound MAM! 2015 MFMER

13 What else is important during your exam? Extracutaneous manifestations in up to 40% Other cutaneous in up to 40% Arthralgias & arthritis Neurologic Ocular Vascular (Raynauds) Lichen sclerosus **More likely in earlier onset disease (before age 10, OR=10) Other autoimmune diseases in 5-8% Vitiligo Alopecia areata JRA Type I IDDM Zulian et al Arthritis Rheumatism MFMER

14 Arthritis in pediatric morphea Arthritis No Arthritis Kashem et al JAAD MFMER

15 Do I need to worry about anything else? 2015 MFMER

16 Likelihood of systemic symptoms Circumscribed ~3% Generalized 15-25% Linear 25-40% Arthralgias Full skin exam including genital exam No labs No biopsy unless uncertain 2015 MFMER

17 Likelihood of systemic symptoms Linear Generalized Circumscribed Evaluate for arthritis Evaluate for other systemic symptoms GI Respiratory Full skin exam, including genital Labs and appropriate consults if systemic symptoms Biopsy usually unnecessary 2015 MFMER

Linear morphea Trunk/limbs ECDS/PRS Arthralgias Arthritis Limb length discrepancy Joint contractures Headache Seizures Ocular Oral Evaluate for

18 Linear morphea Trunk/limbs ECDS/PRS Arthralgias Arthritis Limb length discrepancy Joint contractures Headache Seizures Ocular Oral Evaluate for arthritis Full skin exam, including genital Labs and appropriate consults if systemic symptoms Biopsy usually unnecessary 2015 MFMER

19 Orthopedic complications in pediatric linear morphea 14% required surgical intervention! Schoch et al Ped Dermatol MFMER

20 13 yo F with progressive linear sclerosis 2015 MFMER

21 En coup de sabre morphea/parry-romberg Syndrome 2015 MFMER

$Seizures, ECDS/PRS, and CNS findings 13% have seizures Often refractory to treatment Poor correlation between$

22 Seizures, ECDS/PRS, and CNS findings 13% have seizures Often refractory to treatment Poor correlation between seizures and skin findings Chiu et al Peds Derm Doolittle et al. Neuroradiology MFMER

23 To image or not to image? MRI preferred Poor correlation of CNS findings with skin disease Most commonly bilateral (61%) or ipsilateral (33%) Poor correlation of CNS findings with seizure activity Abnormalities in all with seizures Not all with abnormalities had seizures Our approach = image if CNS symptoms (including headache) Doolittle et al. Neuroradiology MFMER

24 Treatment More aggressive treatment in linear and maybe earlieronset Impact on extracutaneous manifestations unknown Pope and Laxer Pediatr Clin N Am 61(2014) Tollefson et al Peds Derm MFMER

25 Protopic BID Add clobetasol Phototherapy 2015 MFMER

26 Beyond topicals MTX + Steroids MTX + Topicals to select locations Consider adding phototherapy 2015 MFMER

27 When to be aggressive MTX + Steroids (1 mg/kg/day) MTX + Topicals to select locations Continue ~24 months Q6 month monitoring 2015 MFMER

28 Methotrexate in pediatric morphea Various dosing regimens 15 mg/m2/week 0.3 to 1 mg/kg/week Oral vs SQ ~75% respond Significant clinical improvement by ~6 months Well-tolerated Wiebel et al. BJD MFMER

29 Immunizations Update immunizations prior to MTX initiation Utility of serologic titers No live virus vaccines Other vaccines important Influenza Close partnership with pediatrician 2015 MFMER

seizure medication trials MTX 2 seizures/month

30 Ongoing seizures- MJ 2 seizures/yr No seizure medications MTX Prednisone 6 seizures/yr Multiple seizure medication trials MTX 2 seizures/month MTX Seizure meds?need to change MTX 2015 MFMER

31 Beyond methotrexate Mycophenolate mofetil Cyclophosphamide Cyclosporine Tocilizumab 2015 MFMER

32 Pregnancy prevention Pregnancy category X Baseline pregnancy test Consider annual testing Contraception 2015 MFMER

33 How do we do with contraception? Stancil et al. Pediatrics MFMER

34 Who is more likely to get contraception? Stancil et al. Pediatrics MFMER

35 MJ 2015 MFMER

36 Malignancy risk Elandt and Aletaha. Arthritis Research and Therapy MFMER

37 What to expect Mean disease duration = 5 years > 24 months of treatment with MTX with good response associated with increased likelihood of staying in remission Remission less likely with shorter treatment duration Remission less likely if remitting-relapsing course Relapse rate 30-50% in those treated systemically Time to relapse ~16 months (can be decades) Zulian et al JAAD 2012, Pequet et al BJD MFMER

38 Fat grafting 2015 MFMER

39 Kids Kids are not little adults! Linear most common More extracutaneous disease Circumscribed most common More lichen sclerosus Worse QoL scores Adults Lis-Swiety et al JEADV MFMER

40 Cosmetic camouflage Ramien et al. JAAD 2014;71: MFMER

41 Top Diagnosis and activity assessment are still very much clinical 2. Beware the acquired port wine stain 3. Genital exam is important 4. Extracutaneous manifestations more likely in linear subtype and earlier-onset disease 5. Jury is out on baseline CNS imaging in ECDS 6. Not even pediatric dermatologists agree on a topical treatment regimen 7. Systemic treatment usually indicated in linear and generalized subtypes 8. There is a role for fat grafting 9. Prolonged monitoring necessary, especially 1 st two years after treatment discontinuation 10. Remember immunizations and contraception! 2015 MFMER

42 The Dermatology Foundation has supported & advanced my career MFMER

Pediatric Dermatology. Aniza Giacaman. Hospital Universitari Son Espases, Palma de Mallorca

Pediatric Dermatology. Aniza Giacaman. Hospital Universitari Son Espases, Palma de Mallorca Pediatric Dermatology Aniza Giacaman. Hospital Universitari Son Espases, Palma de Mallorca Optimal management of Pediatric Morphea Dr. Yvonne Chiu Pediatric morphea is different from adult morphea More