The Infantile Hemangioma Referral Score: A Validated Tool for Physicians.

OBJECTIVES: Infantile hemangiomas (IHs) are common; some cases require timely referral and treatment to prevent complications. We developed and validated a reliable instrument for timely and adequate referral of patients with IH to experts by nonexpert primary physicians. METHODS: In this multicenter, cross-sectional, observational study, we used a 3-stage process: (1) development of the Infantile Hemangioma Referral Score (IHReS) tool by IH experts who selected a representative set of 42 IH cases comprising images and a short clinical history, (2) definition of the gold standard for the 42 cases by a second independent committee of IH experts, and (3) IHReS validation by nonexpert primary physicians using the 42 gold standard cases. RESULTS: A total of 60 primary physicians from 7 different countries evaluated the 42 gold standard cases (without reference to the IHReS tool); 45 primary physicians evaluated these cases using the IHReS questionnaire, and 44 completed retesting using the instrument. IHReS had a sensitivity of 96.9% (95% confidence interval 96.1%-97.8%) and a specificity of 55.0% (95% confidence interval 51.0%-59.0%). The positive predictive value and negative predictive value were 40.5% and 98.3%, respectively. Validation by experts and primary physicians revealed substantial agreement for interrater reliability and intrarater repeatability. CONCLUSIONS: IHReS, a 2-part algorithm with a total of 12 questions, is an easy-to-use tool for primary physicians for the purpose of facilitating correct and timely referral of patients with IH. IHReS may help practitioners in their decision to refer patients to expert centers.

Effects of intense pulsed light in microstomia in patients with systemic sclerosis: A pilot study.

INTRODUCTION: The effects of intense pulsed light (IPL) on collagen structures are well known in the treatment of photodamaged skin. OBJECTIVE: The objective of this study was to investigate the effect of IPL on sclerotic skin by treating patients with microstomia due to systemic sclerosis. METHODS AND MATERIALS: 13 patients all with microstomia and systemic sclerosis were treated with IPL, PR (530-750 nm filter) and/or VL (555-950 nm filter) applicator. They were treated in the perioral area 8 times with 3-4 weeks of interval and follow-up for 6 months. The outcomes were the inter-incisal distance and the inter-ridge distance. RESULTS: A significant increase in mouth opening of 4.1 mm (95% confidence interval, 1726-6638, p < 0.005) was found in the inter-ridge distance when comparing the distance before treatment with the distance at six-month follow-up. No significant difference was found in the inter-incisal distance. The patients experienced improved mobility and better control of lip movements after the treatments. CONCLUSION: IPL can improve the inter-ridge distance between the lips in patients with microstomia due to systemic sclerosis but does not affect the inter-incisal distance, which is also dependent on the mobility of the mandibular joints. This treatment can be considered an adjunctive therapy in patients with microstomia due to systemic sclerosis.

Heredity of port-wine stains: investigation of families without a RASA1 mutation.

BACKGROUND: The prevalence of capillary malformations, also known as port-wine stains (PWS), is 0.3%. Familial segregation can occur. The capillary malformation-arteriovenous malformation (CM-AVM) phenotype is caused by mutations in the RASA1 gene. In PWS familial cases, the inheritance is considered to be autosomal dominant with variable penetrance. OBJECTIVE: Investigation of the heredity of PWS among patients who attended the vascular anomaly section at the Department of Dermatology in Malmoe, Southern Sweden, between 1993 and 2004 and to study the involvement of the RASA1 gene in patients with a positive family history of PWS. SUBJECTS AND METHODS: A total of 254 patients were examined and given a questionnaire regarding family history of PWS. The first group of 175 patients (109 females and 66 males) reported a negative family history. The other group of 65 patients (46 females and 19 males) reported a positive family history (50% parents or brothers and sisters). RESULTS: The heredity of PWS was 27% (65/240). Twenty-one patients with a positive family history and relatives had no CM-AVM phenotype for mutations in the RASA1 gene. CONCLUSION: PWS may have a stronger heredity component than it was reported earlier and inheritance should be considered when counseling a patient. RASA1 mutations do not explain the PWS in our patients.