Parental Assessment of Infantile Hemangioma Cosmetic Clinical Outcomes: Results of the Spanish Hemangioma Nationwide Prospective Cohort.

BACKGROUND: Functional impairment is the main consideration when it comes to choosing therapy for infantile hemangiomas (IH). However, since most hemangiomas are treated for cosmetic reasons, it is important to know the cosmetic outcome assessed by the parents. OBJECTIVE: To evaluate the aesthetic outcomes of IH, considering the characteristics of the lesions and the treatments used. PATIENTS AND METHODS: The Spanish Infantile Hemangioma Nationwide Prospective Cohort (2016-2022) recruited all consecutive patients diagnosed with IH in 12 Spanish hospitals. The children included had two photos of the IH lesion (at both baseline and at the end of the study). A panel of parents blindly assessed all available photos using a scale from 0 (worst cosmetic outcomes) to 10 (best cosmetic outcomes). The different scores - both before and after treatment - as well as the outcomes percent considered excellent (>9) were described and compared. We analyzed the effect of receiving different therapies and performed causal model analyses estimating the mean treatment effect of parents' assessments. RESULTS: The median follow-up was 3.1 years. A total of 824 photos were evaluated. Baseline aesthetic impact was higher in the propranolol group vs the topical timolol and observation treatment groups (1.85 vs 3.14 vs 3.66 respectively; p<0.001). After treatment, the aesthetic impact was similar between both treatment groups (7.59 vs 7.93 vs 7.90; p>0.2). The causal model could only be applied to the comparison between topical timolol and observation, revealing no differences whatsoever. CONCLUSION: This is the first prospective cohort to analyze the aesthetic outcome of IH. The final aesthetic results of the three therapies were similar, with nearly 40% of patients achieving excellent aesthetic outcomes.

Efficacy and safety of oral sirolimus for high-flow vascular malformations in real clinical practice.

BACKGROUND: Vascular malformations are a complex pathology with few treatment options. In previously published studies, oral sirolimus (rapamycin) has shown promising results in the treatment of low-flow vascular malformations, but its usefulness in high-flow vascular malformations is controversial. AIM: To evaluate the efficacy and safety of sirolimus for the treatment of high-flow vascular malformations in real-life practice. METHODS: In a unit specializing in vascular anomalies, patients treated with oral sirolimus for high-flow vascular malformations were located by consulting the drug dispensations. Reviewing the electronic medical records, data on patient demographics, vascular malformation characteristics, treatments, toxicity and clinical course were collected and statistically analysed. RESULTS: Nine patients with vascular malformations were included: eight had arteriovenous malformation and one had arteriovenous fistula. Six of these malformations were isolated while three were part of a syndrome. Sirolimus was initiated at a dosage of 1-4 mg/day to be taken as a single dose. Partial response was observed in eight of the nine patients (88.9%) with high-flow vascular malformation, while worsening was observed in the remaining patient. The treatment was well tolerated and at the most recent follow-up, five patients remained on treatment with oral sirolimus. CONCLUSION: Our results show that oral sirolimus is a well-tolerated therapeutic option, with an excellent safety profile, which can be useful in the long-term stabilization of patients with high-flow vascular malformations. Single-daily dosage may improve long-term adherence to treatment without worsening its effectiveness.

Safety in Dermatologic Procedures: Ophthalmologic Complications. / Seguridad en procedimientos dermatológicos: complicaciones oftalmológicas.

Ophthalmological complications are uncommon in dermatologic surgery. Nonetheless, all surgeons should know the basics of recognizing, preventing, and treating the 4 complications addressed in this article from the series 'Safety in Dermatologic Procedures'. The first complication that surgeons should be familiar with is eye damage due to chemical irritants. This is a common complication in operating rooms given the presence of irritant substances and the performance of procedures in the eyebrow and eyelid region. The second complication is laser-induced eye damage. In this case, eye protection with safety glasses or eye caps is crucial. The third complication is accidental eyeball perforation, which can occur during certain surgical procedures. The fourth and final complication is retinal artery vasospasm or embolism due to drugs or filler materials. This complication is rare but important to recognize, as early treatment can prevent permanent blindness.

Thermography for the differential diagnosis of vascular malformations.

Vascular malformations classification may pose a diagnostic challenge for physicians. In the early stages, they are diagnosed clinically mainly by visual inspection. For a deeper analysis, Doppler ultrasonography is the preferred technique to determine the haemodynamic behaviour of the anomaly. However, this imaging method is not always available and it requires trained operators to acquire and interpret the images. There is a lack of portable and user-friendly systems that may help physicians in the assessment of vascular malformations. We propose a new diagnostic procedure, more affordable and easier to use, based on a portable thermal camera. This technique provides information about temperature, which has been found to be correlated with the flow rate of the lesion. In our study, > 60 vascular malformations of previously diagnosed patients were analysed with a thermal camera to classify them into low-flow and high-flow malformations. The value was 1 for both sensitivity and specificity of this technique.

Inpatient dermatology consultation in a Spanish tertiary care hospital: a prospective cohort study.

BACKGROUND AND OBJECTIVE: Although dermatology has traditionally been practiced through outpatient consultation, hospitalized patients often have dermatologic problems. The aim of this study was to describe and quantify the volume of interdepartmental consultation of the dermatology department of Hospital Universitario Virgen del Rocío. MATERIAL AND METHODS: In this year-long prospective observational cohort study of 429 patients we used a specific data collection form to record information on consultations for patients admitted between July 2010 and June 2011. RESULTS: The 429 consultations over the 12 months of study accounted for 1.56% of all first consultations with a dermatologist during the period. A mean of 1.75 new patients were attended each day. The mean (SD) age of the patients was 52.63 (19) years, and the ratio of men to women was 1.21. Immunocompromised patients accounted for more than a third of the cohort. The medical specialties consulting most often were internal medicine (n=115) and hematology (n=66). The most frequent diagnoses were inflammatory skin conditions (36.2%) and infectious skin diseases (26.5%). DISCUSSION AND CONCLUSIONS: To date, the role of interdepartmental consultation in dermatology has been little studied. Our results suggest that dermatology plays a fundamental role within the hospital. We have identified the most common skin problems in hospitalized patients and their clinical course.

Prevalence of dystrophic epidermolysis bullosa in Spain: a population-based study using the 3-source capture-recapture method. Evidence of a need for improvement in care.

BACKGROUND: Dystrophic epidermolysis bullosa (DEB) is a rare disease that represents a heavy burden for both the patient and the health care system. There are currently no data on the prevalence of DEB in Spain. OBJECTIVE: To determine the prevalence of DEB in Spain. METHODS: We used data from 3 incomplete population-based sources (hospital dermatology departments, diagnostic laboratories performing antigenic mapping, genetic testing or both, and the Spanish Association of Epidermolysis Bullosa Patients [DEBRA]) and combined them using the 3-source capture-recapture methodology. RESULTS: We identified 152 living DEB patients. The estimated prevalence of DEB was 6.0 cases per million (95% CI, 4.2-11.8) in adults and 15.3 (95% CI, 10.4-40.8) in children under 18 years of age. The data indicated that 77% of the patients were not being followed up in specialized centers of reference; 65% had not had a genetic diagnosis, and 76% were not members of DEBRA. CONCLUSIONS: The prevalence of DEB in Spain is 6.0 patients per million (95% CI, 4.2-11.8), a figure higher than previous estimates in many areas, but similar to those found in other southern Europe countries. The north-south difference may represent real geographic differences in prevalence, but it might be due to the fact that most of the data come from registries with a lower than expected catchment. Many patients are not being followed up in centers of reference, do not have genetic diagnosis, and are not members of patients' associations, suggesting that there is room for considerable improvement in their care.

[Propranolol for the treatment of severe hemangiomas of infancy: results from a series of 28 patients]. / Tratamiento con propranolol oral para hemangiomas infantiles graves: serie de 28 pacientes.

BACKGROUND AND OBJECTIVES: Recent reports have described the successful use of propranolol to treat severe hemangiomas of infancy. The few case series that have been reported, however, have included only a small number of patients. The aim of this study was to describe the results of oral propranolol treatment for severe hemangiomas of infancy in terms of treatment outcome and the occurrence of adverse events. PATIENTS AND METHODS: A descriptive, observational study was undertaken in a series of children with severe hemangiomas of infancy seen in the pediatric dermatology unit at Hospital Universitario Virgen del Rocío in Seville, Spain between July 2008 and December 2009. Patients were included if they had hemangiomas in the proliferative phase or involuting lesions with substantial residual deformity. All children were treated with oral propranolol (2 mg/kg/d) and followed until September 2010. Epidemiologic characteristics were analyzed along with treatment response at 3, 6, 9, 12, and 18 months; adverse events were also recorded at those times. RESULTS: Thirty-six hemangiomas were treated in 28 patients. Propranolol treatment was effective in all cases, with a good or complete response in 88.2% at 6 months. Effects were apparent within a few hours of treatment, which was effective in both growing and involuting hemangiomas. In ulcerated hemangiomas, the mean healing time was 61 days. Adverse events were mild and self-limiting. Only 2 patients discontinued treatment due to hypotension. CONCLUSIONS: In the majority of cases, oral propranolol produced rapid and sustained improvements in hemangiomas of infancy and shortened the natural course of the disease with few side effects. However, no significant reductions in symptoms or healing time were observed in ulcerated hemangiomas.

Baseline description of the Spanish Academy of Dermatology infantile haemangioma nationwide prospective cohort. Comparison of patients treated with propranolol in routine clinical practice with previous pivotal clinical trial data. / Descripción basal de la cohorte prospectiva de hemangioma infantil a nivel nacional de la Asociación Española de Dermatología. Comparación de los pacientes tratados con propranolol en la práctica clínica rutinaria y los datos de un ensayo clínico pivotal previo.

BACKGROUND: There are several therapeutic options for infantile haemangiomas (IH). Propranolol is used according to a pivotal trial. We aimed to describe the characteristics of IH in clinical practice, including the therapies used, and to compare the characteristics of patients treated with propranolol with those of the trial to assess its external validity. METHODS: Consecutive patients attending 12 Spanish hospitals from June 2016 to October 2019 were included (n=601). RESULTS: The mean age was 3.9 (SD:1.9) months, with a 2:1 female-to-male ratio. Most IHs were localized (82%, 495), superficial (64%, 383) and located in the face (25%, 157) and trunk (31%, 188). Median size was 17 (IR: 10-30) x 12 (IR: 7-20) mm. Complications were found in 16 (3%) patients. Treatment was initiated for 52% (311). Most patients received timolol (76%, 237); propranolol was reserved for complications or high-risk IHs. Aesthetic impairment was the main reason for starting therapy (64%, 199). Several characteristics of the patients and IHs treated with propranolol are similar to those of the pivotal clinical trial, but 1/3 of IHs did not reach the minimum diameter to meet the inclusion criteria, and important prognostic information was not reported. CONCLUSIONS: As most patients receive treatment for aesthetic impairment, there is a need to better understand the aesthetic results of therapies and to increase evidence on the use of timolol, which is currently the most common therapy. Propranolol is being used in a population generally similar to that of the trial; however, this statement cannot be definitely confirmed.

[PHACES syndrome]. / Síndrome de PHACES.

INTRODUCTION: PHACES syndrome associates a segmental facial hemangioma with cerebral malformations, aortic branches/cranial arteries anomalies, cardiac defects, eye anomalies or ventral wall defects. The aim of this study is to analyze our experience with this syndrome. MATERIAL AND METHODS: Retrospective study of the cases seen at our unit in the last year. RESULTS: We treat 4 cases; 3 girls and 1 child. Besides the segmental hemangioma they presented: 3 vascular cerebral malformations; 2 structural cardiopathies; 2 cerebral malformations, 1 microftalmia. We did not find ventral wall defects. A case received treatment with two cycles of metilprednisolone i.v. and oral prednisone, with favourable course; two cases received initial treatment with oral prednisone continued of oral propanolol in rising pattern up to 2 mg/kg/day, Obtaining both the detention of the tumour growth and regression of the lesion, with very good tolerance. A 7-year-old patient has been treated with colouring pulse laser for her residual lesions. CONCLUSIONS: When we see a segmental facial hemangioma we must perform a wide diagnostic study in order to discard a PHACES syndrome. Multidisciplinar approach to the patient by a wide expert's group gets an earlier diagnose and improves the outcome. Propranolol is a promising therapeutic alternative.

Attendee Survey and Practical Appraisal of a Telegram®-Based Dermatology Congress During the COVID-19 Confinement. / Congreso virtual de dermatología realizado a través de Telegram® durante el confinamiento de la COVID-19: organización y evaluación.

INTRODUCTION: The COVID-19 pandemic outbreak introduced dramatic changes in all our lives, daily practice, and medical conferences. In search of a tool to spread dermatologic knowledge during confinement, an online medical meeting was held on April 25th to 26th, 2020. In this study, we aimed to assess the characteristics, opinion and satisfaction of the attendees to a free-of-charge online congress. Secondarily, we intended to explain how this meeting was prepared. MATERIAL AND METHODS: Online survey administered to the attendees to an online congress organised via the Telegram® Messenger App. Its organisation and planning, which needed no financial support and was done by volunteer organisers, moderators and speakers, is described step by step. RESULTS: The satisfaction of both speakers and attendees was very high. All participants considered that this format had a great present and future, and most of them rated it as superior to regular face-to-face meetings. Female gender and predominantly private practice favoured this opinion. DISCUSSION: The COVID-19 pandemic has forced the cancellation of most scientific gatherings. This has been seen by some authors as an excellent opportunity, encouraging medical societies and organisations to lead the change to virtual meetings. Although confinement did not allow real contact, our online meeting showed it was possible to ensure interaction and participation between attendees, moderators and speakers. Dermatologists enjoyed some dermatologic science, even despite the extraordinary circumstances disrupting their daily clinical practice. Most of them felt they were participating in something new and compelling that many felt superior to traditional meetings.

[Paediatric dermatology emergencies in a tertiary hospital]. / Urgencias dermatológicas pediátricas en un hospital de tercer nivel.

INTRODUCTION: In recent years, the increasing demand for dermatological consultations in the Emergency department has resulted in the publication of a variety of studies on this subject. However, most of them deal with the general population, without taking into account the changes in frequencies found in young children (ages 0-14). OBJECTIVES: To determine the frequency of various dermatological diagnoses made by the on-call paediatrician in the Emergency Department, and after referral to Paediatric Dermatology. MATERIAL AND METHODS: Firstly, a descriptive retrospective study was performed that included all patients aged between 0 and 14 years old who were seen after being referred to the emergency paediatric dermatologist by the on-call paediatrician from June 2010 to December 2013. Secondly, an analytical study was carried by calculating the kappa index calculus, in order to establish the diagnostic concordance between the emergency paediatrician and the paediatric dermatologist. RESULTS: A total of 861 patients, with a mean age of 4.5 years were included. More than half of the skin disorders analysed were eczema (27%) and infections (26%). The 5 main diagnoses were: atopic dermatitis (16%), acute prurigo simplex (5%), tinea (5%), pyogenic granuloma (4%), and molluscum contagiosum (4%). Additional tests were only required in 16% of the cases. The kappa index obtained was 0.206 (95% CI: 0.170-0.241). CONCLUSIONS: The dermatology consultations in the Emergency Department were shown to be frequent and mostly involved minor diseases. Collaboration between paediatricians and dermatologists resulted in a high treatment success rate, leading to a low percentage of additional tests required and a high rate of discharges.

Successful management of Churg-Strauss syndrome using omalizumab as adjuvant immunomodulatory therapy: first documented pediatric case.

Churg-Strauss syndrome (CSS) is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis; it is extremely rare in childhood and defined according to the Chapel-Hill Consensus as an eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels. Children commonly have a history of asthma and sinusitis whilst clinical presentation typically involves pulmonary tract and less frequently skin, heart, gastrointestinal tract, and peripheral nerves. Cardiopulmonary disease is higher in children and prognosis is worse. It is associated with significant eosinophilia and raised serum IgE-levels. ANCA are only found in 25% of childhood cases. Here we report the case of a 10-year-old girl who presented to us with vomiting, abdominal pain, and weight loss, paresthesias of lower extremities and breathlessness as well as a history of asthma, sinusitis and allergic rhinitis. She was treated with corticosteroids, cyclophosphamide, intravenous immunoglobulin, mycophenolate mofetil (MMF), and rituximab. However, remission was only achieved after initiation of omalizumab therapy, a recombinant humanized anti-IgE antibody. To the best of our knowledge this is the first pediatric patient suffering from CSS successfully managed with adjuvant anti-IgE therapy resulting in the control of respiratory as well as gastrointestinal symptoms.

Bacillary angiomatosis with atypical clinical presentation in an immunocompetent patient.

Bacillary angiomatosis is a recently described infectious disease that usually affects immunosupressed hosts with a previous history of contact with cats. We report a rare case of bacillary angiomatosis in an immunocompetent 59-year-old woman with no history of previous exposure to cats, and atypical clinical features (fever and subcutaneous nodules with ulceration on the left ankle). Histopathology of the lesion showed extensive ulceration and reactive tumor-like vascular proliferation of the blood vessels with swollen endothelial cells and an inflammatory infiltrate including neutrophils and lymphocytes in the dermis and subcutis. Staining with the Warthin-Starry method demonstrated the presence of clustered bacilli located in the extracellular matrix adjacent to the proliferating endothelial cells. Diagnosis was confirmed with the detection of Bartonella spp. DNA in the affected skin and in bone marrow using polymerase chain reaction.