Wynnis L. Tom Editor Severe Skin Diseases in Children Beyond Topical Therapy 123 Severe Skin Diseases in Children Wynnis L. Tom Editor Severe Skin Diseases in Children Beyond Topical Therapy Editor Wynnis L. Tom Pediatric and Adolescent Dermatology University of California, San Diego and Rady Children’s Hospital San Diego, CA USA ISBN 978-3-642-39531-4 ISBN 978-3-642-39532-1 (eBook) DOI 10.1007/978-3-642-39532-1 Springer Heidelberg New York Dordrecht London Library of Congress Control Number: 2013954925 © Springer-Verlag Berlin Heidelberg 2014 This work is subject to copyright. 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Printed on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com) Pref ace Treating skin disease in children has its challenges, with fewer approved agents and supportive studies, additional time over which risks and side effects must be considered, and variable family dynamics that may affect our approach. Nevertheless, such hurdles must not be left untackled, as many children suffer from severe conditions with a signifi cant impact on daily life and, at times, on growth and development. The fi eld of pediatric dermatology is moving forward in big strides, utiliz- ing collaborative research efforts to generate advances and a stronger evi- dence basis for care. But as one author aptly put it, at present our approaches are more art than science, and I greatly thank all the contributors to this text for being willing to share their experience and insights on management. It is by group effort that we have put together a current portrait of systemic and other non-topical treatment options for the conditions we encounter. I myself have learned many pearls in completing this project and hope that you, the reader, experience the same. San Diego, CA, USA Wynnis L. Tom v Acknowledgements My biggest gratitude is again to my colleagues in the fi eld for dedicating time to make this effort possible. Our patients deserve the highest recognition, for teaching us valuable lessons and inspiring us to strive to do better on their behalf. I also thank Springer, including Dr. Sverre Klemp, for recognizing the importance of providing guidance in the management of children with severe skin conditions and Ellen Blasig and Ioanna Panos for their editorial and developmental assistance. I am grateful to my mentors in dermatology and pediatric dermatology at the University of California, San Diego; Rady Children’s Hospital, San Diego; and Saint Louis University, who fostered my desire and abilities to utilize systemic therapies. Finally, my family and friends, who are my pillars of support. vii Contents Part I Infl ammatory Diseases 1 Atopic Dermatitis . 3 David A. Dasher and Wynnis L. Tom 2 Psoriasis and Pityriasis Rubra Pilaris . 21 Omar Pacha, Megan J. Schlichte, and Adelaide A. Hebert 3 Acne: Vulgaris and Variants . 37 Marissa J. Perman and Anne W. Lucky 4 Hidradenitis Suppurativa . 53 Melinda Jen and Mary Wu Chang Part II Immune-Mediated/Autoimmune Diseases 5 Autoimmune Bullous Diseases of Childhood . 67 Nicole A. Weitz, Emily M. Mintz, and Kimberly D. Morel 6 Connective Tissue Disease . 91 Yvonne E. Chiu, Sheetal S. Vora, Ronald M. Laxer, and Elena Pope 7 Autoinfl ammatory Syndromes . 123 Tien V. Nguyen and Kieron S. Leslie Part III Vascular Lesions and Tumors 8 Infantile Hemangiomas . 143 Denise W. Metry 9 Complex Vascular Malformations . 157 Diana Camarillo, Christopher P. Hess, and Ilona J. Frieden 10 Other Vascular Tumors . 173 Tina S. Chen and Wynnis L. Tom ix x Contents Part IV Genodermatoses 11 Current and Novel Approaches for Genetic Skin Disorders . 189 Joey E. Lai-Cheong and Amy S. Paller 12 Multisystem Management of Epidermolysis Bullosa. 203 Meena R. Julapalli and Anna L. Bruckner Part V Other Growths and Tumors 13 Lymphoproliferative Disorders . 217 Christian L. Baum and Dawn M. Davis 14 Pediatric Mastocytosis . 229 Julianne Mann, Sabra Leitenberger, and Alfons Krol Index . 241 P a r t I I n fl ammatory Diseases Atopic Dermatitis 1 David A. Dasher and Wynnis L. Tom Key Points (i.e., convenience of administration, • Phototherapy can be considered fi rst in availability), side effect profi le, and those with diffuse atopic dermatitis impact on other medical treatments. (AD) uncontrolled by topical therapies, • Given current therapies are mainly but it may not always be feasible or immunosuppressive agents, discussion effective as well. of risks versus benefi ts is critical before • The main systemic agents used at this initiation, and once adequate response is time for severe, recalcitrant AD are achieved, systemic treatments should be cyclosporine, azathioprine, mycopheno- weaned off as tolerated. late mofetil, and methotrexate. • Systemic steroids are limited by their side effects and mainly should be reserved for short courses to bridge to another agent; intravenous immuno- Abbreviations globulin and interferon- gamma are alternatives for those failing the above ACD Allergic contact dermatitis traditional systemic agents and/or with AD Atopic dermatitis frequent infections. HSTCL Hepatosplenic T-cell lymphoma • Considerations in choice of agent: effi - IVIg Intravenous immunoglobulin cacy, time to effect, cost, ease of use MED Minimal erythema dose MM Mycophenolate mofetil PUVA Psoralen with ultraviolet A TPMT Thiopurine methyltransferase Atopic dermatitis (AD, commonly called “eczema”) has been increasing in prevalence over D. A. Dasher , MD time and currently affects about 20 % of children Alamance Dermatology , 1638 Memorial Drive , in developed countries [ 1 ]. While the majority is Burlington , NC 27215 , USA adequately managed using topical treatment regi- W . L . T o m , M D ( *) mens, a small group of patients continue to have Depatments of Pediatrics and Medicine severe, persistent disease. Phototherapy is one (Dermatology) , University of California, San Diego therapeutic option that can be used, but may not and Rady Children’s Hospital, San Diego , 8010 Frost Street, Suite 602, San Diego , CA 92123 , USA always be feasible or effective as well. Systemic e-mail: [email protected] agents may be appropriate for such cases, but W.L. Tom (ed.), Severe Skin Diseases in Children, 3 DOI 10.1007/978-3-642-39532-1_1, © Springer-Verlag Berlin Heidelberg 2014 4 D.A. Dasher and W.L. Tom their use does require careful thought and appeared to be short-lived to 2–3 months on discussion. discontinuation, suggesting that UVA1 treat- ment may be best suited as a bridge to another long- term maintenance therapy. The authors con- 1.1 Phototherapy for Atopic cluded that UVB, particularly narrow-band UVB Dermatitis (nbUVB, 311 nm), was more useful in manage- ment of chronic AD. nbUVB is less erythemo- The observation of clinical improvement during genic and generally more effective at clearing summer months prompted clinicians to utilize skin disease relative to broadband UVB (bbUVB, phototherapy, the controlled exposure of skin to 290–320 nm), although some have found it at light, as a potentially viable treatment for AD times to be more irritating [ 2 , 4 ]. Two subsequent patients with persistent, widespread disease. comparatives studies, however, found that UVA1 Various wavelengths, particularly within the was comparable to nbUVB for chronic, moderate ultraviolet (UV) spectrum, have shown benefi t to severe AD [ 5 , 6 ]. Unfortunately, none of the for AD [ 2 ]. The presumed mechanism of action above RCTs included children. is the suppression of pro-infl ammatory cytokines Subsequently, Clayton et al. [ 7 ] reported and molecules with induction of lymphocyte complete clearance or minimal residual activity apoptosis and, additionally, inhibition of in 40 % of 50 children age 4–16 years with Langerhans cells and keratinocytes (in the case of severe AD who received at least ten exposures UVB) and dermal fi broblasts, dendritic cells, of nbUVB. Those with higher MEDs were more mast cells, and granulocytes (in the case of UVA, likely to clear. More recently, Pavlovsky et al. which penetrates more deeply) [ 3 ]. With the [ 8 ] reported 75 % or greater improvement in exception of oral psoralen given with UVA 69 % of 36 children with mean age of 13 years (PUVA), phototherapy has the advantage of being treated with nbUVB for an average of an externally administered treatment, and thus 3.3 months. Overall, nbUVB is the preferred avoids the risk of systemic toxicities.