A PHACES syndrome unmasked by propranolol interruption in a tetralogy of Fallot patient: case report and extensive review on new indications of beta blockers.

Infantile hemangiomas (IHs) are the most common benign tumors of infancy and usually they don't require specific therapy. In 10-20% of cases IHs are able to generate complication and medical/surgical intervention is needed. For many decades standard treatment consisted in oral or intralesional corticosteroids until Leaute-Labreze and colleagues published the first report on the efficacy of propranolol for cutaneous infantile hemangiomas in 2008. IHs can be sometimes part of complex syndrome. Here we report the case of a patient with tetralogy of Fallot operated at 5 month of age who stopped propranolol treatment for hypoxic spells and unusually developed facial and subglottic IHs configuring the diagnosis of PHACES syndrome (posterior fossa brain malformations, hemangioma, arterial anomalies, cardiac defects and/or aortic coarctation, ocular anomalies and sternal defects). To our knowledge this is the first report in the international literature of a delayed appearance of an infantile hemangioma involving the skin and the airways (PHACES syndrome). The pathophysiological explanation relies on the mechanism of action of propranolol which seems to act initially with vasoconstriction, down-regulating proangiogenetic factors and inducing endothelial cell apoptosis. Many decades since their introduction ß-blockers are useful in a growing group of diseases. The pleiotropic effect of ß-adrenoceptors antagonists is not yet deeply understood, residing in neurohormonal regulation systems and angiogenesis and proving to be an effective treatment from cardiovascular to oncological illnesses.

Clinical effectiveness of a special silk textile in the treatment of recurrent pediatric inflammatory vulvitis: an open label pilot study.

AIM: Pediatric inflammatory vulvitis (PIV) is clinically characterized by itching, soreness and inflammation and can be due to both an infective process and a non-specific irritative process, especially in atopic patients. Sometimes these non-specific PIVs, that tend to be recurrent, can be overinfected, with exacerbation of the clinical features. The importance of the cleansers, emollients, and the kind of textiles that enter in direct contact all day long with the inflamed skin, is well known. The study objective is the evaluation of the safety and efficacy of the transpiring, slightly elastic knitted silk briefs, with anti-bacterial and non-irritating properties, registered as Dermasilk®, in recurrent PIV. METHODS: The study we conducted was a prospective cohort study of 12 pre-pubertal girls, aged between 2 and 10 years, affected by recurrent PIV, that used Dermasilk® briefs in association to conventional treatments. RESULTS: Dermasilk® briefs have proven to be an effective and safe adjuvant product available for use in association with conventional drugs for the treatment of recurrent PIV. CONCLUSION: Dermasilk® briefs play an important role in the management of the flares of recurrent PIV, proven by an earlier resolution of symptoms, as well as in the maintenance of the remission and in the prevention of overinfections.

Discoid chronic lupus erythematosus at the site of a previously healed cutaneous leishmaniasis: an example of isotopic response.

The term "isotopic response" describes the occurrence of a new skin disorder at the site of another, unrelated and already healed one. We report here the case of a 38-year-old woman who referred to us for an infiltrated, red-brownish plaque localized on her left cheek. The patient had been treated for a cutaneous leishmaniasis, confirmed by the histologic examination, localized at the same site. She was completely healed after an appropriate local and systemic treatment. She experienced the occurrence of the new plaque at the site of the previously healed cutaneous leishmaniasis three month later. Histologic examination and laboratory tests were consistent with a diagnosis of discoid cutaneous chronic lupus erythematosus. Treatment with hydroxychloroquine, topical clobetasol and topical tretinoin resulted in flattening and clearing of the lesion. Our case is the first case of isotopic response where a discoid chronic lupus erythematosus had occurred at the site of an already healed cutaneous leishmaniasis. We speculate that the activation of type-1 interferon system may be involved in the pathogenesis of our case.

The effect of summer holidays and sun exposure on atopic dermatitis.

AIM: The aim of this study was to investigate the effect of sunlight during summer holidays and to assess the seasonability of atopic dermatitis in 43 consecutive patients with mild-moderate atopic dermatitis referring to the Pediatric Outpatient Clinic of the University of Bologna. METHODS: Patients had to answer an open questionnaire. RESULTS: The collected data showed that 74.4% of the patients affected by mild-moderate atopic dermatitis had complete resolution during summer holidays, 16.3% had improvement and only 9.3% had no modification of atopic dermatitis severity, confirming the seasonability of the disease, with improvement during summertime and worsening in the other seasons. Seaside holidays produced a significantly greater improvement than mountains holidays, with complete resolution of the disease in 91.2% versus 11.1% of patients, P<0.01. Conclusion. These data support the hypothesis on the positive effect of UV radiation on atopic dermatitis in patients without eczema after local therapy with corticosteroids or immunomodulators, but are in contrast with those reported by other authors from Northern Europe. This discrepancy is probably due to the latitude and different climate. In the Mediterranean area and in southern locations greater improvements are observable.

Usefulness of dermatoscopy for the early diagnosis of sebaceous naevus and differentiation from aplasia cutis congenita.

In early infancy, a congenital area of alopecia of the scalp may be the clinical presentation of various diseases, with the two most common being sebaceous naevus and aplasia cutis congenita. Typical lesions are easily diagnosed, but soon after birth, the differential diagnosis may be difficult if the clinical features of the lesion are not distinctive. In this paper, we report the usefulness of dermatoscopy in such cases.

The cardiovascular response to underwater pedaling at different intensities: a comparison of 4 different water stationary bikes.

AIM: Among the fitness activities, cycling in water immersion performed with water stationary bikes (WSB) has become very popular. However, there is a lack of information concerning their physiological demand. The aim of the present study was to assess the cardiovascular responses to four WSBs. METHODS: Twenty-two healthy participants underwent a graded exercise test on a cycle ergometer. Sixteen of them, 8 males (age 31.5 + or - 8.2 years) and 8 females (age 31.9 + or - 5.8 years) with the same level of peak oxygen consumption (VO(2peak)) were chosen and submitted, in random order, to four incremental testing sessions performed with four different models of WSB, one with no resistance (WSB1), two with resistance added to the bottom bracket axle (WSB2 and WSB3), one with resistance added to the pedals (WSB4). The VO(2peak) and heart rate peak (HR(peak)) were measured in all five conditions. RESULTS: Since no significant main effect for gender was detected, the data were grouped and analyzed all together. No significant differences were found in VO(2peak) and HR(peak) within the five testing conditions, whilst time to exhaustion (P<0.01) and pedaling frequency (RPM) at volitional exhaustion (P<0.01) were both significantly different across the four WSBs. When pedaling at 70 RPM, the oxygen consumption and heart rate response (as % of VO(2peak) and HR(peak)) were approximately 45% and 60% for WSB1, 60% and 70% for WSB3, 90% and 90% for WSB2 and WSB4, respectively. CONCLUSIONS: The present results provide useful information for instructors and practitioners because they demonstrate that different models of WSBs could elicit very different cardiovascular responses.

Pitfalls in the diagnosis of airport malaria. Seven cases observed in the Paris area in 1994.

Clinical and biological pitfalls that lead to incorrect or delayed diagnoses of airport malaria are described based on 7 cases reported from the Paris region in the summer of 1994. We also report the outcome and the epidemiological features of these patients.