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Summary Background Oral propranolol is widely prescribed as first-line treatment for infantile haemangiomas (IHs). Anecdotally, prescribing practice differs widely between centres. Objectives The Propranolol In the Treatment of Complicated Haemangiomas (PITCH) Taskforce was founded to establish patterns of use of propranolol in IHs. Methods Participating centres entered data on all of their patients who had completed treatment with oral propranolol for IHs, using an online data capture tool. Results The study cohort comprised 1097 children from 39 centres in eight European countries. 76·1% were female and 92·8% had a focal IH, with the remainder showing a segmental, multifocal or indeterminate pattern. The main indications for treatment were periocular location (29·3%), risk of cosmetic disfigurement (21·1%) and ulceration and bleeding (20·6%). In total 69·2% of patients were titrated up to a maintenance regimen, which consisted of 2 mg kg−1 per day (85·8%) in the majority of cases. 91·4% of patients had an excellent or good response to treatment. Rebound growth occurred in 14·1% upon stopping, of whom 53·9% were restarted and treatment response was recaptured in 91·6% of cases. While there was no significant difference in the treatment response, comparing a daily maintenance dose of < 2 mg kg−1 vs. 2 mg kg−1 vs. > 2 mg kg−1, the risk of adverse events was significantly higher: odds ratio (OR) 1 vs. adjusted OR 0·70, 95% confidence interval (CI) 0·33–1·50, P = 0·36 vs. OR 2·38, 95% CI 1·04–5·46, P = 0·04, Ptrend < 0·001. Conclusions The PITCH survey summarizes the use of oral propranolol across 39 European centres, in a variety of IH phases, and could be used to inform treatment guidelines and the design of an interventional study.

Summary Aquagenic palmar wrinkling (APW) is characterized by the rapid and transient oedematous wrinkling of the palms after brief immersion in water. APW has been associated with cystic fibrosis (CF). Since the discovery of the CF gene, the clinical spectrum of CF has broadened from classic severe CF to include milder ‘atypical CF’ and ‘CF-related disorders’. We report an unusual case in which APW occurred in a patient with no lung disease, and in whom investigations showed evidence of CF gene dysfunction. APW may be a presenting feature of a CF-related disorder and should prompt investigation of CF gene dysfunction.

Summary Background There is only retrospective evidence for the efficacy of narrowband ultraviolet B (NB-UVB) in children with eczema. Objectives To measure the difference in means for objective scores [Six Area Six Sign Atopic Dermatitis score (SASSAD), percentage surface area] and quality-of-life scores, between patients treated with NB-UVB and unexposed cohorts at the end of treatment, and 3 and 6 months post-treatment. Methods Twenty-nine children aged 3–16 years for whom NB-UVB was indicated, were scored prospectively using SASSAD and percentage surface area involvement at baseline, at 12 weeks (end of treatment) and 3 and 6 months post-NB-UVB. Their scores were compared with those of unexposed children (n = 26) for whom NB-UVB phototherapy was indicated and offered, but who chose not to undertake treatment. Results There was a 61% reduction in mean SASSAD score in the NB-UVB cohort compared with an increase of 6% in the unexposed cohort. Mean SASSAD score for the NB-UVB cohort at the end of treatment was 11·6 vs. 24·8 for the unexposed; difference in means −13·2 [95% confidence interval (CI) −18·7 to −7·7, P

Blister formation and eccrine sweat gland necrosis have been recognized to occur in states of impaired consciousness and were first reported following barbiturate intoxication. Their etiology is complex and cannot simply be explained by pressure effects. Now that barbiturates are less frequently used, clinicians are likely to be less aware of the phenomenon of coma blister formation; however, newer drugs have also been associated with the occurrence of coma blisters. We describe 2 new associations of coma blisters and anticonvulsants in children. In the first child, blisters recurred on multiple occasions along with obtundation and edema. Our aims are to alert clinicians to the occurrence of coma blisters in children sedated on anticonvulsant medications and to report the new finding of recurrent coma blisters.

Clinical dermatologists have great difficulty keeping abreast of research in genetic skin disease. This is because there is too much information, in too many places, and in an unfamiliar language. In this review we have simplified and tabulated our current knowledge of the genodermatoses. We hope this 'at a glance' online guide will help dermatologists apply research findings to clinical practice and give informed advice to patients.

Naevoid basal cell carcinoma syndrome (NBCCS) or Gorlin syndrome is a rare autosomal dominant cancer disorder. The Gorlin's gene, Patched 1 (PTCH1), maps to Chromosome 9q. Germline mutations of PTCH1 occur in patients with NBCCS. The subsequent loss of the remaining allele results in cancer formation. We present a patient with NBCCS and additional phenotypic features including severe developmental delay, short stature and hypotelorism who was found to have an interstitial chromosome 9q deletion. The NBCCS phenotype in our patient occurred as a result of PTCH1 deletion in contrast with an inherited mutation of this gene.

Maximal static inspiratory pressure (P(IMAX)) generated at the mouth is one of the tests of inspiratory muscle strength in children. In adults, inspiratory muscle strength has also been assessed using sniff nasal inspiratory pressure (SNIP). This test is easier to perform than P(IMAX) and might therefore be applicable to children. To test this hypothesis, we measured P(IMAX) and SNIP in 116 children aged 4 to 11 years (54 girls, 62 boys). P(IMAX) was measured using a tube mouthpiece and SNIP using a tightly fitting plug in one nostril, while a sniff was performed through the other nostril. Both tests were performed from functional residual capacity (FRC). Pressure was measured with a differential pressure transducer and displayed in real time on a computer screen. Weight, standing height, sitting height, gender, and age were recorded. There was a significant difference (P0.01) in group mean (SD) data between SNIP (81.3 (27.4) cmH(2)O) and P(IMAX) (67.9 (28.1) cmH(2)O). Bland/Altman analysis demonstrated a mean difference of -13.5 cmH(2)O (SD 21.4) between the techniques. Regression analysis indicated highly significant relations (P0.01) between SNIP and P(IMAX), and between weight, standing and sitting height, and age for SNIP, and between weight, standing height, and age for P(IMAX). SNIP and P(IMAX) were greater in boys than girls (83.2 vs. 79.2 cmH(2)O SNIP; 72.9 vs. 62.0 cmH(2)O P(IMAX)), but this difference was only significant for P(IMAX) (P0.05). SNIP was significantly greater than P(IMAX) (P0.01) in both boys and girls. These data suggest that SNIP provides a simple, noninvasive additional test to P(IMAX) for assessing inspiratory muscle strength in children.

Personal interviews concerning health beliefs and behaviors were conducted with a parent and child in each of 250 households. Index scores were constructed for parental and child health beliefs, and these scores were entered, along with demographic variables, in a series of multiple regression analyses predicting child health beliefs and behaviors. The age of the child was the variable most highly associated with three of four child health behaviors and four of six child health beliefs. The children's snacking between meals and cigarette smoking were related to several parental behaviors and, to a lesser extent, parental health beliefs. The children's health beliefs were less predictable than were their health behaviors, and the observed significant relationships were with parental health beliefs and demographics. The implications for the design of health education programs are discussed.