abSTRaCT
REviEw
Skin changes are common in children. Common concerns are birthmarks (e.g., hemangiomas and port wine stains), atopic and contact dermatitis, acne, and alopecia areata. The authors review advances in common and not so common skin changes in pediatric patients. J Clin Aesthet Dermatol. 2017;10(3 Suppl1):S8–S15
Pediatric Dermatology a
LESLIE CASTELO-SOCCIO, MD, PhD, and aPATRICK MCMAHON, MD The Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania a
CUTANEOUS PROBLEMS OCCUR frequently in children; up to 30 percent of pediatric primary care visits involve a skin-related problem.1–4 Common among these problems are atopic dermatitis, seborrheic dermatitis, contact dermatitis, and acne. Yet pediatric dermatology, first recognized in 2000 as a boarded subspecialty of dermatology, is in its infancy.5 Despite the demand, there is a national shortage of pediatric dermatologists, which has made it difficult to impart adequate dermatology training for pediatricians. For this reason, many dermatologists, pediatricians, and primary care physicians manage the pediatric population when it comes to dermatology issues. Education for clinicians is imperative to meet the burden of pediatric dermatology cases, many of which can be highly complex. The aim of this review is to discuss emerging concerns in pediatric dermatology.
C
Infantile hemangiomas (IH), common tumors in infants with a prevalence around five percent, may be treated in several ways.6 More than half of IH present in the head and neck region (60%).7 It is crucial first to INFANTILE HEMANGIOMAS
differentiate between a superficial hemangioma, which may respond to topical therapy, and a deep hemangioma, which is typically treated with oral propranolol. Timolol 0.5%, a gel-forming solution, is the first-line topical treatment for superficial IH.8 Early treatment is highly recommended. Propranolol, a beta-adrenergicblocker, has emerged as a preferred treatment option for complicated IH cases.9,10 As a beta-blocker, propranolol may be associated with changes in the heart rate or rhythm, including symptomatic bradycardia.11 The use of propranolol therapy for IH may vary from institution to institution. In a multiinstitutional survey of treatment practices for IH (n=18 respondents, 15 institutions), respondents at 67 percent of institutions said they routinely consulted with cardiology colleagues before initiating propranolol therapy, and the median dosage of propranolol hydrochloride to start therapy was 2.00mg/kg/d±1.65mg/kg/d (range 0.45– 2.50mg/kg/d). Treatment duration ranged from 4 to 8 months (33%) or 8 to 12 months (67%), and the decision to discontinue therapy was based on clinical response (50%) or patient’s age (43%).12 While guidelines have been
Disclosures: The authors have no conflicts of interest relevant to the content of this article. Author correspondence: Dr. Leslie Castelo-Soccio; email: [email protected]
S8
JCAD
JOURNAL OF CLINICAL AND AESTHETIC DERMATOLOGY March 2017 • Vol. 10 • No. 3 • Supplement 1
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established for the initiation, treatment indications, and clinical monitoring of propranolol therapy for IH, the role of electrocardiography (ECG) testing remains unclear. There is no consensus as to whether it is required for pretreatment evaluation. The use of routine ECG testing was evaluated in a twocenter study of 162 patients who were examined prior to the planned commencement of propranolol treatment for IH.13 In this study, 43 percent of patients who underwent routine ECG monitoring obtained abnormal results. This, in turn, led to 28 formal consultations with pediatric cardiologists, but none of those “abnormal” results were ultimately precluded from propranolol therapy. Moreover, no patient in this study experienced any adverse effects from propranolol therapy that could have been predicted by an ECG. Thus, the role of ECG monitoring in this context remains unclear, but this evidence suggests that ECG testing may not be necessary.14 For patients with bradycardia or a history of arrhythmias, features of posterior fossa brain malformations (PHACE syndrome), familial history of early cardiac death or congenital heart disease, or maternal history of connective tissue disease, an ECG may still be helpful and appropriate. Thus, the potential benefits of an ECG should be weighed against its cost and potential utility. PORT WINE STAINS AND STURGEWEBER SYNDROME
It has long been thought that the JCAD
distribution of port wine stains (PWS) follows the trigeminal nerve, but new evidence has found a relation to genetic mosaicism.15 PWS on the face, which appear to follow the embryonic vasculature (rather than the trigeminal nerve), may be an isolated finding or may occur along with Sturge-Weber Syndrome (SWS). Today, it is more useful to consider embryologic segments when discussing PWS rather than the older V1, V2, V3 criteria. SWS may be diagnosed when the patient presents with malformations of the cerebral and ocular vascular systems along with facial PWS. In a study of 192 children with facial PWS, two predictors of adverse outcomes emerged: a PWS involving any part of the forehead (defined by the line joining the outer canthus of the eye to the top of the ear, including the upper eyelid) and an abnormal magnetic resonance imaging (MRI) scan. Thus, it may be important for pediatric patients presenting with PWS on the forehead to undergo an ophthalmology review and a brain MRI.16 PWS and SWS are thought to be somatic mosaic mutations that disrupt vascular development. It has been theorized that the development time at which these mutations occur defines the severity and extent of PWS and SWS. In a study of wholegenome sequencing of DNA, a nonsynonymous single-nucleotide variant in GNAQ was identified in affected tissue samples of 88 percent of participants with SWS and 92 percent of patients with non-SWS
PWS, but not in any of the affected tissue of others with unrelated cerebrovascular malformations or control patients. The mutant allele was prevalent in affected tissue at rates of 1.0 to 18.1 percent.17 In a study of 192 pediatric patients with facial PWS, adverse outcomes (defined as seizures, abnormalities in neurodevelopment, glaucoma, and abnormal MRI scans) were calculated. Based on the Fisher’s exact p-value, patients with forehead plaques were significantly more likely to have seizures, abnormal neurodevelopment, and glaucoma (p
REviEw
Skin changes are common in children. Common concerns are birthmarks (e.g., hemangiomas and port wine stains), atopic and contact dermatitis, acne, and alopecia areata. The authors review advances in common and not so common skin changes in pediatric patients. J Clin Aesthet Dermatol. 2017;10(3 Suppl1):S8–S15
Pediatric Dermatology a
LESLIE CASTELO-SOCCIO, MD, PhD, and aPATRICK MCMAHON, MD The Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania a
CUTANEOUS PROBLEMS OCCUR frequently in children; up to 30 percent of pediatric primary care visits involve a skin-related problem.1–4 Common among these problems are atopic dermatitis, seborrheic dermatitis, contact dermatitis, and acne. Yet pediatric dermatology, first recognized in 2000 as a boarded subspecialty of dermatology, is in its infancy.5 Despite the demand, there is a national shortage of pediatric dermatologists, which has made it difficult to impart adequate dermatology training for pediatricians. For this reason, many dermatologists, pediatricians, and primary care physicians manage the pediatric population when it comes to dermatology issues. Education for clinicians is imperative to meet the burden of pediatric dermatology cases, many of which can be highly complex. The aim of this review is to discuss emerging concerns in pediatric dermatology.
C
Infantile hemangiomas (IH), common tumors in infants with a prevalence around five percent, may be treated in several ways.6 More than half of IH present in the head and neck region (60%).7 It is crucial first to INFANTILE HEMANGIOMAS
differentiate between a superficial hemangioma, which may respond to topical therapy, and a deep hemangioma, which is typically treated with oral propranolol. Timolol 0.5%, a gel-forming solution, is the first-line topical treatment for superficial IH.8 Early treatment is highly recommended. Propranolol, a beta-adrenergicblocker, has emerged as a preferred treatment option for complicated IH cases.9,10 As a beta-blocker, propranolol may be associated with changes in the heart rate or rhythm, including symptomatic bradycardia.11 The use of propranolol therapy for IH may vary from institution to institution. In a multiinstitutional survey of treatment practices for IH (n=18 respondents, 15 institutions), respondents at 67 percent of institutions said they routinely consulted with cardiology colleagues before initiating propranolol therapy, and the median dosage of propranolol hydrochloride to start therapy was 2.00mg/kg/d±1.65mg/kg/d (range 0.45– 2.50mg/kg/d). Treatment duration ranged from 4 to 8 months (33%) or 8 to 12 months (67%), and the decision to discontinue therapy was based on clinical response (50%) or patient’s age (43%).12 While guidelines have been
Disclosures: The authors have no conflicts of interest relevant to the content of this article. Author correspondence: Dr. Leslie Castelo-Soccio; email: [email protected]
S8
JCAD
JOURNAL OF CLINICAL AND AESTHETIC DERMATOLOGY March 2017 • Vol. 10 • No. 3 • Supplement 1
REviEw
established for the initiation, treatment indications, and clinical monitoring of propranolol therapy for IH, the role of electrocardiography (ECG) testing remains unclear. There is no consensus as to whether it is required for pretreatment evaluation. The use of routine ECG testing was evaluated in a twocenter study of 162 patients who were examined prior to the planned commencement of propranolol treatment for IH.13 In this study, 43 percent of patients who underwent routine ECG monitoring obtained abnormal results. This, in turn, led to 28 formal consultations with pediatric cardiologists, but none of those “abnormal” results were ultimately precluded from propranolol therapy. Moreover, no patient in this study experienced any adverse effects from propranolol therapy that could have been predicted by an ECG. Thus, the role of ECG monitoring in this context remains unclear, but this evidence suggests that ECG testing may not be necessary.14 For patients with bradycardia or a history of arrhythmias, features of posterior fossa brain malformations (PHACE syndrome), familial history of early cardiac death or congenital heart disease, or maternal history of connective tissue disease, an ECG may still be helpful and appropriate. Thus, the potential benefits of an ECG should be weighed against its cost and potential utility. PORT WINE STAINS AND STURGEWEBER SYNDROME
It has long been thought that the JCAD
distribution of port wine stains (PWS) follows the trigeminal nerve, but new evidence has found a relation to genetic mosaicism.15 PWS on the face, which appear to follow the embryonic vasculature (rather than the trigeminal nerve), may be an isolated finding or may occur along with Sturge-Weber Syndrome (SWS). Today, it is more useful to consider embryologic segments when discussing PWS rather than the older V1, V2, V3 criteria. SWS may be diagnosed when the patient presents with malformations of the cerebral and ocular vascular systems along with facial PWS. In a study of 192 children with facial PWS, two predictors of adverse outcomes emerged: a PWS involving any part of the forehead (defined by the line joining the outer canthus of the eye to the top of the ear, including the upper eyelid) and an abnormal magnetic resonance imaging (MRI) scan. Thus, it may be important for pediatric patients presenting with PWS on the forehead to undergo an ophthalmology review and a brain MRI.16 PWS and SWS are thought to be somatic mosaic mutations that disrupt vascular development. It has been theorized that the development time at which these mutations occur defines the severity and extent of PWS and SWS. In a study of wholegenome sequencing of DNA, a nonsynonymous single-nucleotide variant in GNAQ was identified in affected tissue samples of 88 percent of participants with SWS and 92 percent of patients with non-SWS
PWS, but not in any of the affected tissue of others with unrelated cerebrovascular malformations or control patients. The mutant allele was prevalent in affected tissue at rates of 1.0 to 18.1 percent.17 In a study of 192 pediatric patients with facial PWS, adverse outcomes (defined as seizures, abnormalities in neurodevelopment, glaucoma, and abnormal MRI scans) were calculated. Based on the Fisher’s exact p-value, patients with forehead plaques were significantly more likely to have seizures, abnormal neurodevelopment, and glaucoma (p
