Faculty Bibliography

OBJECTIVE: To characterize the atypical cutaneous presentations in the coxsackievirus A6 (CVA6)-associated North American enterovirus outbreak of 2011-2012. METHODS: We performed a retrospective case series of pediatric patients who presented with atypical cases of hand, foot, and mouth disease (HFMD) from July 2011 to June 2012 at 7 academic pediatric dermatology centers. Patients were included if they tested positive for CVA6 or if they met clinical criteria for atypical HFMD (an enanthem or exanthem characteristic of HFMD with unusual morphology or extent of cutaneous findings). We collected demographic, epidemiologic, and clinical data including history of skin conditions, morphology and extent of exanthem, systemic symptoms, and diagnostic test results. RESULTS: Eighty patients were included in this study (median age 1.5 years, range 4 months-16 years). Seventeen patients were CVA6-positive, and 63 met clinical inclusion criteria. Ninety-nine percent of patients exhibited a vesiculobullous and erosive eruption; 61% of patients had rash involving >10% body surface area. The exanthem had a perioral, extremity, and truncal distribution in addition to involving classic HFMD areas such as palms, soles, and buttocks. In 55% of patients, the eruption was accentuated in areas of eczematous dermatitis, termed "eczema coxsackium." Other morphologies included Gianotti-Crosti-like (37%), petechial/purpuric (17%) eruptions, and delayed onychomadesis and palm and sole desquamation. There were no patients with serious systemic complications. CONCLUSIONS: The CVA6-associated enterovirus outbreak was responsible for an exanthem potentially more widespread, severe, and varied than classic HFMD that could be confused with bullous impetigo, eczema herpeticum, vasculitis, and primary immunobullous disease.

To systematically review the literature evaluating efficacy and adverse events of propranolol treatment for infantile hemangiomas, we searched the MEDLINE and Cochrane databases for all studies examining the response of infantile hemangiomas (IHs) to propranolol published between June 12, 2008, and June 15, 2012. Forty-one studies with 1,264 patients were included; 74% of patients were female and approximately 30% had received other treatments before propranolol. Propranolol was initiated at a mean age of 6.6 months at a mean dose of 2.1 mg/kg/day and for a mean treatment duration of 6.4 months. The response rate for patients with IHs treated with propranolol was 98% (range 82%-100%), with response rate defined as any improvement with propranolol. Treatment response rates were comparable for studies evaluating IHs at specific sites, such as periorbital IHs. Studies that followed patients after treatment completion reported IH rebound growth in 17% of patients. There were 371 adverse events reported in 1,189 patients. The most common adverse events were changes in sleep (n = 136) and acrocyanosis (n = 61). Serious adverse events were rare, with reports of symptomatic hypotension in five patients, hypoglycemia in four, and symptomatic bradycardia in one. This systematic review of 1,264 patients treated with propranolol for IHs showed a high rate of efficacy and a low rate of serious adverse events.

BACKGROUND: Subcutaneous fat necrosis (SCFN) of the newborn is a form of panniculitis that affects full-term neonates who often have suffered either birth asphyxia or hypothermia. The induction of hypothermia in newborns is becoming frequently used to reduce the neurologic sequelae associated with birth asphyxia. The risk of SCFN in neonates undergoing this therapy is unknown. Observation We describe a neonate who developed an abscess-like presentation of SCFN and subsequent asymptomatic hypercalcemia after undergoing whole-body cooling for hypoxic-ischemic encephalopathy. CONCLUSIONS: Hypothermia protocols may be placing newborns at increased risk for the development of SCFN. Clinicians should recognize this association, and newborns who undergo therapeutic cooling should have frequent dermatologic assessments.

BACKGROUND AND PURPOSE: We present neuroradiologic findings in 17 patients with posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal or ventral defects (PHACES) association and identify those at highest risk of central nervous system (CNS) structural, cerebrovascular, and neurodevelopmental abnormalities. MATERIALS AND METHODS: Patients with PHACES association were identified in the Vascular Anomalies Program at New York University Medical Center from 1998 to 2007. Many patients were followed in conjunction with other specialists at the Birthmark Institute at Roosevelt Hospital. Clinical records and imaging studies were reviewed retrospectively. Criteria for diagnosis of PHACES were based on previously published indicators. Imaging studies were independently re-reviewed by a neuroradiologist. Segmental mapping of cutaneous hemangioma distribution by photograph review and presence or absence of other PHACES-associated findings were correlated with radiologic findings. RESULTS: Patients with large facial cutaneous (S1-S4) hemangiomas were especially at risk of CNS structural and cerebrovascular anomalies; S1 with ocular anomalies; and S3 with airway, ventral, and cardiac anomalies. All patients with CNS structural malformations had a cerebrovascular abnormality, and this cohort was at risk for developmental and/or other neurologic sequelae. Four patients had supratentorial CNS anomalies, including cortical dysgenesis and migration abnormalities. Some patients with CNS arteriopathy progressed to aneurysms. CONCLUSION: Our data support and expand the work of others, identifying risk factors for segmental hemangiomas. In addition to posterior fossa CNS anomalies, supratentorial anomalies may be present in patients with PHACES, and this may correlate with significant clinical sequelae. The long-term prognosis of these patients remains unknown

The purpose of this study was to evaluate the effectiveness of a 2-component intervention for biological and foster parent (pairs) to improve parenting practices, co-parenting, and child externalizing problems. Participants were biological and foster parents (N=128) of primarily neglected children (ages 3 to 10 years) placed in regular foster homes. Biological and foster parents were randomly assigned in pairs to the intervention (n=80) or a usual care (n=48) condition. Intervention families received a 12-week parenting course (Incredible Years) and a newly developed co-parenting component. Key findings included significant gains in positive parenting and collaborative co-parenting for both biological and foster parents at the end of the intervention. At follow-up, intervention parents sustained greater improvement in positive parenting, showed gains in clear expectations, and reported a trend for fewer child externalizing problems. Findings supported the feasibility of offering joint parenting training to meet the needs of participating families and demonstrated that the co-parenting construct applied to families in the foster care system was amenable to intervention