F. Boralevi

Propranolol for Severe Hemangiomas of Infancy

Despite their self-limited course, infantile capillary hemangiomas can impair vital or sensory functions or cause disfigurement. These authors have observed in 11 children that propranolol can inhi...

Propranolol for Severe Infantile Hemangiomas: Follow-Up Report

OBJECTIVE: Infantile hemangiomas (IHs) are the most-common soft-tissue tumors of infancy. We report the use of propranolol to control the growth phase of IHs. METHODS: Propranolol was given to 32 children (21 girls; mean age at onset of treatment: 4.2 months) after clinical and ultrasound evaluations. After electrocardiographic and echocardiographic evaluations, propranolol was administered with a starting dose of 2 to 3 mg/kg per day, given in 2 or 3 divided doses. Blood pressure and heart rate were monitored during the first 6 hours of treatment. In the absence of side effects, treatment was continued at home and the child was reevaluated after 10 days of treatment and then every month. Ultrasound measurements were performed after 60 days of treatment. RESULTS: Immediate effects on color and growth were noted in all cases and were especially dramatic in cases of dyspnea, hemodynamic compromise, or palpebral occlusion. In ulcerated IHs, complete healing occurred in <2 months. Objective clinical and ultrasound evidence of longer-term regression was seen in 2 months. Systemic corticosteroid treatment could be stopped within a few weeks. Treatment was administered for a mean total duration of 6.1 months. Relapses were mild and responded to retreatment. Side effects were limited and mild. One patient discontinued treatment because of wheezing. CONCLUSION: Propranolol administered orally at 2 to 3 mg/kg per day has a consistent, rapid, therapeutic effect, leading to considerable shortening of the natural course of IHs, with good clinical tolerance.

A Randomized, Controlled Trial of Oral Propranolol in Infantile Hemangioma

BACKGROUND Oral propranolol has been used to treat complicated infantile hemangiomas, although data from randomized, controlled trials to inform its use are limited. METHODS We performed a multicenter, randomized, double-blind, adaptive, phase 2-3 trial assessing the efficacy and safety of a pediatric-specific oral propranolol solution in infants 1 to 5 months of age with proliferating infantile hemangioma requiring systemic therapy. Infants were randomly assigned to receive placebo or one of four propranolol regimens (1 or 3 mg of propranolol base per kilogram of body weight per day for 3 or 6 months). A preplanned interim analysis was conducted to identify the regimen to study for the final efficacy analysis. The primary end point was success (complete or nearly complete resolution of the target hemangioma) or failure of trial treatment at week 24, as assessed by independent, centralized, blinded evaluations of standardized photographs. RESULTS Of 460 infants who underwent randomization, 456 received treatment. On the basis of an interim analysis of the first 188 patients who completed 24 weeks of trial treatment, the regimen of 3 mg of propranolol per kilogram per day for 6 months was selected for the final efficacy analysis. The frequency of successful treatment was higher with this regimen than with placebo (60% vs. 4%, P<0.001). A total of 88% of patients who received the selected propranolol regimen showed improvement by week 5, versus 5% of patients who received placebo. A total of 10% of patients in whom treatment with propranolol was successful required systemic retreatment during follow-up. Known adverse events associated with propranolol (hypoglycemia, hypotension, bradycardia, and bronchospasm) occurred infrequently, with no significant difference in frequency between the placebo group and the groups receiving propranolol. CONCLUSIONS This trial showed that propranolol was effective at a dose of 3 mg per kilogram per day for 6 months in the treatment of infantile hemangioma. (Funded by Pierre Fabre Dermatologie; ClinicalTrials.gov number, NCT01056341.).

Cutaneous and Ocular Signs of Childhood Rosacea

UNLABELLED Objectives To describe the clinical features of cutaneous and ocular manifestations of childhood rosacea, to propose diagnostic criteria, and to emphasize the possible severity of ocular complications in this age group. DESIGN Retrospective study. SETTING Tertiary referral center. Patients Children aged 1 to 15 years who had received a diagnosis of cutaneous and/or ocular rosacea and were seen between January 1, 1996, and December 31, 2005. RESULTS Of 20 patients, 11 had ocular and cutaneous rosacea, 6 had isolated cutaneous involvement, and 3 had isolated ocular involvement. Dermatologic examination results were sufficient to diagnose rosacea in 12 of the patients (60%). The most common presentation was a papulopustular eruption on a telangiectatic background. In 11 patients (55%), ocular involvement preceded the skin eruption. Among the ophthalmologic manifestations, chalazions and blepharoconjunctivitis were the main presenting symptoms; keratitis was observed in 4 patients and corneal ulcers in 2. Ten patients were treated with oral metronidazole. Intermittent treatment for at least 3 months was used to avoid neurologic toxic effects and to achieve complete remission. Conclusion Although rare, childhood rosacea should be recognized because of the possible severity of ocular involvement.

Idiopathic facial aseptic granuloma: a multicentre prospective study of 30 cases.

Background  Idiopathic facial aseptic granuloma (IFAG) was recently described in a single‐centre retrospective study as a skin condition that occurs specifically in childhood.

Multivariate analysis of factors associated with early-onset segmental and nonsegmental vitiligo: a prospective observational study of 213 patients.

Background  Although mixed forms have been described recently, segmental (SV) and nonsegmental vitiligo (NSV) are considered as clinically distinct. However, limited epidemiological data are available to help distinguish associated factors, and recent genome‐wide association studies have been restricted to NSV. The higher prevalence of SV in children is helpful when comparing the two major presentations of the disease.

A prospective study of risk for Sturge-Weber syndrome in children with upper facial port-wine stain

BACKGROUND Upper facial port-wine stain (PWS) is a feature of Sturge-Weber syndrome (SWS). Recent studies suggest that the distribution of the PWS corresponds to genetic mosaicism rather than to trigeminal nerve impairment. OBJECTIVES We sought to refine the cutaneous distribution of upper facial PWS at risk for SWS. METHODS This was a prospective multicenter study of consecutive cases of upper facial PWS larger than 1 cm² located in the ophthalmic division of trigeminal nerve distribution in infants aged less than 1 year, seen in 8 French pediatric dermatology departments between 2006 and 2012. Clinical data, magnetic resonance imaging, and photographs were systematically collected and studied. PWS were classified into 6 distinct patterns. RESULTS In all, 66 patients were included. Eleven presented with SWS (magnetic resonance imaging signs and seizure). Four additional infants had suspected SWS without neurologic manifestations. Hemifacial (odds ratio 7.7, P = .003) and median (odds ratio 17.08, P = .008) PWS patterns were found to be at high risk for SWS. A nonmedian linear pattern was not associated with SWS. LIMITATIONS Small number of patients translated to limited power of the study. CONCLUSIONS Specific PWS distribution patterns are associated with an increased risk of SWS. These PWS patterns conform to areas of somatic mosaicism. Terminology stipulating ophthalmic division of trigeminal nerve territory involvement in SWS should be abandoned.

Poor long term outcome of severe alopecia areata in children treated with high dose pulse corticosteroid therapy

SIR, Alopecia areata (AA) occurring before puberty is associated with a poorer prognosis than adult AA. It is more likely to have a chronic evolution and to worsen over time. Treatment with boluses of methylprednisolone (MPBT) is currently used to treat this T-cell mediated auto-immune disease. It is considered as an effective and well-tolerated treatment for extensive patchy AA. However, despite the tendency for a chronic evolution of AA occurring before puberty, long-term assessment of this therapy to the best of our knowledge has not been reported. The objective of this cross sectional study was to assess the long-term effectiveness of MPBT on severe AA occurring before puberty. The 12 consecutive patients aged between 1 and 16 years who had been treated with MPBT between January 1999 and December 2005 were asked to come for a visit. The treatment was done according to Kiesch et al. All patients were systematically examined before and one month after the end of the therapy. Long-term assessment was carried out between January 2006 and March 2007. The short term and long term outcomes were measured according to the following criteria: good if regrowth was better than 60%, moderate if between 30 and 60% and bad below 30%, when compared to baseline photographs obtained at the first MBPT course. Patients’ data are summarized in Table 1. The short-term effectiveness of the treatment was assessed as good for seven children of 12, moderate for three and bad for two. The median long term follow-up after treatment was 42 months (12–76 months). The long-term outcome was good for one child (median follow-up 48 months), moderate for three children (median follow-up 29 months) and bad for eight (median follow-up 50 months). Five children of 12 had either AA totalis or universalis at the reassessment visit. The median time to relapse was 9 months (2–58 months). No side effect of MBPT was noticed in the 12 children. Despite the aggressive type of AA and long disease duration before treatment (median 12 months) of the patients included, our retrospective analysis confirms MBPT effectiveness at short term assessments. In our short series, the long duration of the disease before treatment does not seem to influence the short-term outcome. Nevertheless, this cross sectional study suggests that the effectiveness of MPBT is not maintained at longer term. Five of the 12 children had either AA totalis or AA universalis at the long-term assessment visit. These results corroborate a recent long term follow-up study showing the poor outcome of severe AA occurring during childhood. Moreover, our results are similar to the first long-term assessment of topical immunotherapy in children, with only 2 of 33 children who maintained complete hair regrowth after a 10 years follow up. Thus, the long-term evolution of severe AA treated with MPBT or other therapeutic regimen reflects mostly the spontaneous evolution of severe AA. Pulsed systemic corticosteroid therapy modifies the initial course of severe AA but does not generally influence the long-term outcome. The long term responders are unfortunately not recognizable at initial examination. Those findings are important for the initial counselling of our

Autoimmune thyroid disease in vitiligo: multivariate analysis indicates intricate pathomechanisms

Background  Vitiligo/nonsegmental vitiligo (NSV) is often associated with thyroid dysimmunity although very few reports have studied this association using multivariate logistic regression.

Clinical phenotype of scabies by age.

OBJECTIVE: Scabies has a clinical presentation that seems to vary according to age. We conducted a prospective study with the goal of delineating the clinical presentation of the disease into 3 groups of age: infants, <2 years; children, 2 to 15 years; and adults, >15 years. METHODS: This trial was a prospective, multicenter observational study in consecutive patients with a confirmed diagnosis of scabies who were seen in 13 French Departments of Dermatology and Pediatric Dermatology between April 2010 and April 2011. A standardized questionnaire was completed for each patient. To identify factors associated with patient age, comparisons between the 3 age groups were conducted by using univariate and multivariate multinomial logistic regression analyses. RESULTS: A total of 323 individuals were included; the gender ratio (female:male) was 1.2:1. In univariate analysis, infants were more likely to have facial involvement. In multivariate logistic regression, relapse was more frequent in children (odds ratio [OR]: 2.45 [95% confidence interval (CI):1.23–4.88]) and infants (OR: 3.26 [95% CI: 1.38–7.71]). In addition, family members with itch (OR: 2.47 [95% CI: 1.04–5.89]), plantar (OR: 20.57 [95% CI: 7.22–58.60]), and scalp (OR: 16.94 [95% CI: 3.70–77.51]) involvement were also found to be independently associated with the age group <2 years. CONCLUSIONS: There is a specific clinical presentation of scabies in infants and children. Taking into account these specificities may be helpful for the early diagnosis and the identification of cases to prevent the propagation of the disease.