FAT1, which encodes a protocadherin, is one ofthe most frequently mutated genes in human cancers(1-5). However, the role and the molecular mechanisms by which FAT1 mutations control tumour initiation and progression are poorly understood. Here, using mouse models of skin squamous cell carcinoma and lung tumours, we found that deletion of Fat1 acceleratestumour initiation and malignant progression and promotes a hybrid epithelial-to-mesenchymal transition (EMT) phenotype. We also found this hybrid EMT state in FAT1-mutated human squamous cell carcinomas. Skin squamous cell carcinomas in which Fat1 was deleted presented increased tumour stemness and spontaneous metastasis. We performed transcriptional and chromatin profiling combined with proteomic analyses and mechanistic studies, which revealed that loss of function of FAT1 activates a CAMK2-CD44-SRC axis that promotes YAP1 nuclear translocation and ZEB1 expression that stimulates the mesenchymal state. This loss of function also inactivates EZH2, promotingSOX2expression, which sustains the epithelial state. Our comprehensive analysis identified drug resistance and vulnerabilities in FAT1-deficient tumours, which have important implications for cancer therapy. Our studies reveal that, in mouse and human squamous cell carcinoma, loss of function of FAT1 promotes tumour initiation, progression, invasiveness, stemness and metastasis through the induction of a hybrid EMT state.

Randomized studies to assess the efficacy of Mohs micrographic surgery in basal cell and squamous cell carcinomas are limited by methodological and ethical issues and a lack of long follow-up periods. This study presents the real-life results of a nationwide 7-years cohort on basal cell carcinoma and squamous cell carcinoma treated with Mohs micrographic surgery. A prospective cohort was conducted in 22 Spanish centres (from July 2013 to February 2020) and a multivariate analysis, including characteristics of patients, tumours, surgeries and follow-up, was performed. A total of 4,402 patients followed up for 12,111 patientyears for basal cell carcinoma, and 371 patients with 915 patient-years of follow-up for squamous cell carcinoma were recruited. Risk factors for recurrence included age, non-primary tumours and more stages or unfinished surgeries for both tumours, and immunosuppression for squamous cell carcinoma. Incidence rates of recurrence were 1.3 per 100 person-years for basal cell carcinoma (95% confidence interval 1.1-1.5) and 4.5 for squamous cell carcinoma (95% confidence interval 3.3-6.1), being constant over time (0-5 years). In conclusion, follow-up strategies should be equally intense for at least the first 5 years, with special attention paid to squamous cell carcinoma (especially in immunosuppressed patients), elderly patients, non-primary tumours, and those procedures requiring more stages, or unfinished surgeries.

Purpose: The aim of this study was to evaluate the outcomes, complications and psychosocial impact of surgical treatment of giant congenital melanocytic nevus (GCMN). Methods: Patients with surgically treated GCMN who attended our clinic between May 2014 and May 2018 were included. Patient demographics and data on the characteristics of the nevus, surgical treatment, and the psychosocial impact (including C-DLQI/DLQI questionnaires) were collected. Results: One hundred thirty-six patients were included (median age 9 years). Mean age at first surgery was 34 (+/- 61.45) months; 5.53 (+/- 3.69) surgical interventions were necessary to completely excise the nevus. The expanded skin flap was the preferred surgical technique in most locations. Complications were common but not severe. Of the patients studied, 70.4% reported that the surgery had a minor impact on their quality of life (QoL). Patients and caregivers stated that surgical treatment should begin as soon as possible, even in cases where early treatment did not have an impact on their QoL nor on their satisfaction with the surgery (p < 0.05). The lower the patient age at first surgery, the higher the surgeon's satisfaction (p < 0.01). Conclusions: Surgical treatment is a safe option for management of GCMN, and has a low impact on QoL. Patients, caregivers, and surgeons agree that the treatment should begin as soon as possible. This is the largest single-center study evaluating surgical treatment in GCMN patients and its psychosocial impact, and the first to take into account the patient, caregivers and dermatologists opinion of surgical results. (C) 2021 Elsevier Inc. All rights reserved.

Purpose: The aim of this study was to evaluate the outcomes, complications and psychosocial impact of surgical treatment of giant congenital melanocytic nevus (GCMN). Methods: Patients with surgically treated GCMN who attended our clinic between May 2014 and May 2018 were included. Patient demographics and data on the characteristics of the nevus, surgical treatment, and the psychosocial impact (including C-DLQI/DLQI questionnaires) were collected. Results: One hundred thirty-six patients were included (median age 9 years). Mean age at first surgery was 34 (+/- 61.45) months; 5.53 (+/- 3.69) surgical interventions were necessary to completely excise the nevus. The expanded skin flap was the preferred surgical technique in most locations. Complications were common but not severe. Of the patients studied, 70.4% reported that the surgery had a minor impact on their quality of life (QoL). Patients and caregivers stated that surgical treatment should begin as soon as possible, even in cases where early treatment did not have an impact on their QoL nor on their satisfaction with the surgery (p < 0.05). The lower the patient age at first surgery, the higher the surgeon's satisfaction (p < 0.01). Conclusions: Surgical treatment is a safe option for management of GCMN, and has a low impact on QoL. Patients, caregivers, and surgeons agree that the treatment should begin as soon as possible. This is the largest single-center study evaluating surgical treatment in GCMN patients and its psychosocial impact, and the first to take into account the patient, caregivers and dermatologists opinion of surgical results.

Background and objective: Basal cell carcinoma (BCC) is the most common skin cancer in the general population. BCC is managed in a variety of ways, and available international guidelines are difficult to put into practice in Spain. This guideline aims to improve the management of BCC based on current evidence and provide a point of reference for Spanish dermatologists. Material and methods: Members of the Spanish Oncologic Dermatology and Surgery Group (GEDOC) with experience treating BCC were invited to participate in drafting this guideline. The drafters used the ADAPTE collaboration process to develop the new guideline based on existing ones, first summarizing the care pathway and posing relevant clinical questions. They then searched for guidelines, assessed them with the AGREEII (Appraisal of Guidelines for Research and Evaluation) tool, and searched the selected guidelines for answers to the clinical questions. Finally, the recommendations were drafted and submitted for external review. Results: The highest-scoring guidelines were from the Association of Dermatologists, the National Comprehensive Cancer Network, the European Dermatology Forum, and the European Academy of Dermatology and Venereology. A total of 11 clinical questions were answered. Conclusions: This new guideline answers the working group's clinical questions about the routine management of BCC in Spain. It provides dermatologists with a tool they can use for decision-making while taking into consideration the resources available and patient preferences.

Background: Organ transplant recipients (OTR) have a higher risk of actinic keratosis (AK) and keratinocyte carcinomas (KC). There are no clinical trials assessing the effectiveness of daylight photodynamic therapy (DPDT) to prevent new AK and KC in OTR. Objectives: To determine whether repeated treatments of field cancerization with DPDT are effective in preventing new AK and KC in OTR. Methods: A randomized, intra-subject controlled, evaluator-blind, split-face and/or scalp trial, from April 2016 to October 2018. Participants were OTR older than 18 years, 1-year posttransplant, with at least 5 AK on each hemi-face/hemi-scalp. One side received six field treatments with DPDT: two sessions 15 days apart at baseline, two at 3 months and two at 9 months after baseline. Control side received lesion-directed treatment with cryotherapy (double freeze-thaw) at baseline, 3 and 9 months. Total number of lesions (AK and KC) at 21 months, number of new AK and KC at 3, 9, 15 and 21 months and treatment preferences were analysed. Results: Of 24 men included, 23 were analysed at 3 months; and 21, at 9, 15 and 21 months. Mean (SD) age was 69.8 years (9.2). The total number of lesions at 21 months was 4.7 (4.3) for DPDT and 5.8 (5.0) for control side; P = 0.09. DPDT showed significantly lower means [SD] of new lesions compared to control side at 3 months (4.2 [3.4] vs. 6.8 [4.8]; P < 0.001), 9 months (3.0 [3.3] vs. 4.3 [3.4]; P = 0.04) and 15 months (3.0 [4.6] vs. 4.8 [5.0]; P = 0.02), and non-significant at 21 months (3.7 [3.5] vs. 5.0 [4.5]; P = 0.06). Most participants preferred DPDT. Conclusion: DPDT showed potential effectiveness in preventing new AK and KC in OTR by consecutive treatments of field cancerization. The preference for DPDT could facilitate adherence to the long-term treatment necessary in these patients.

Various techniques have been described for the reconstruction of surgical defects of the upper lip after skin cancer. Here, we propose a technique for reconstructing medium or large defects located at the junction of the nasogenian and nasolabial folds with a hatchet flap. We report on three patients treated with this technique, describing the surgical procedure and some interesting caveats. Two patients reconstructed with direct closure in this location are described in order to emphasize the better aesthetic result achieved with the hatchet flap, especially in terms of symmetry. While direct closure tends to decrease the ipsilateral nasolabial fold, the hatchet flap extends the scar to the fold below the commissure in a natural wrinkle, becoming symmetrical and much less noticeable after a few months. We conclude that the hatchet flap is technically easy and rapidly implemented for defects in the apical triangle of the upper lip, and achieves better results in terms of facial symmetry than direct closure.

Background Reconstruction of large auricular defects with full-thickness skin grafts (FTSG) is a commonly reported option, but less attention has focused on the advantages and indications of using split-thickness skin grafts (STSG) in the ear. Objective We sought to report our experience using STSG for repair of defects located on the auricular concave surfaces, highlighting the utility of choosing the adjacent hairy skin as donor site. Methods We performed a retrospective review of all Mohs micrographic defects on the auricular concave surfaces repaired with STSG obtained from the adjacent hairy skin, between January 2017 and July 2018 at our institution. Results A total of 16 patients with defects on the auricular concavities resulting from removal of non-melanoma skin cancer were reconstructed with STSG taken from the adjacent hairy skin. Only one patient experienced partial graft failure and no other complications were observed after 6-month follow-up. Conclusion Split-thickness skin grafts are suitable for reconstructing concave areas in the ear, providing good cosmetic results with a simple, cost-effective and easily reproducible technique. Choosing the adjacent hairy skin as a donor area shortens the operative and postoperative time, and allows the procedure to be performed in a single surgical field.

Background The use of Mohs micrographic surgery (MMS) for rare cutaneous tumors is poorly defined. We aim to describe the demographics, tumor presentation and topography, surgery characteristics and complications of MMS for rare cutaneous tumors in a national registry. Methods Prospective cohort study of patients treated with MMS in Spain between July 2013 and June 2018. The inclusion criteria were patients with cutaneous tumors with final diagnosis different from basal cell carcinoma, squamous cell carcinoma, dermatofibrosarcoma protuberans, or any kind of melanoma. Results Five thousand and ninety patients were recorded in the registry, from which only 73 tumors (1.4%) fulfilled the inclusion criteria: atypical fibroxanthoma (18), microcystic adnexal carcinoma (10), extramammary Paget's disease (7), Merkel cell carcinoma (5), dermatofibroma (4), trichilemmal carcinoma (4), desmoplastic trichoepithelioma (4), sebaceous carcinoma (3), leiomyosarcoma (2), porocarcinoma (2), angiosarcoma (2), trichoblastoma (1), superficial acral fibromyxoma (1), and others (10). No intra-surgery morbidity was registered. Postsurgery complications appeared in six patients (9%) and were considered mild. Median follow-up time was 0.9 years during which three Merkel cell carcinomas, one angiosarcoma, one microcystic adnexal carcinoma, and four others recurred (12.3%). Conclusion This national registry shows that rare cutaneous tumors represent a negligible part of the total MMS performed in our country with a low complication rate.

Background The clinical and pathological features of primary melanoma are not sufficiently sensitive to accurately predict which patients are at a greater risk of relapse. Recently, a 31-gene expression profile (DecisionDx-Melanoma) test has shown promising results. Objectives To evaluate the early prognostic performance of a genetic signature in a multicentre prospectively evaluated cohort. Methods Inclusion of patients with AJCC stages IB and II conducted between April 2015 and December 2016. All patients were followed up prospectively to assess their risk of relapse. Prognostic performance of this test was evaluated individually and later combined with the AJCC staging system. Prognostic accuracy of disease-free survival was determined using Kaplan-Meier curves and Cox regression analysis. Results of the gene expression profile test were designated as Class 1 (low risk) and Class 2 (high risk). Results Median follow-up time was 26 months (IQR 22-30). The gene expression profile test was performed with 86 patients; seven had developed metastasis (8.1%) and all of them were in the Class 2 group, representing 21.2% of this group. Gene expression profile was an independent prognostic factor for relapse as indicated by multivariate Cox regression analysis, adjusted for AJCC stages and age. Conclusions This prospective multicentre cohort study, performed in a Spanish Caucasian cohort, shows that this 31gene expression profile test could correctly identify patients at early AJCC stages who are at greater risk of relapse. We believe that gene expression profile in combination with the AJCC staging system could well improve the detection of patients who need intensive surveillance and optimize follow-up strategies.

Atopic dermatitis (AD) is a chronic cutaneous inflammatory disorder, characterized by skin barrier disruption. Dermacare is a new cosmetic formulation, which enhances moisturization, reinforces and repairs the skin barrier, and prevents cutaneous microbiota imbalance. To demonstrate its safety and efficacy, a prospective, open-label, and multicenter study was carried out on patients diagnosed with mild to moderate AD. Transepidermal water loss (TEWL), clinical severity, Desquamation Index, Patient/Investigator Global Assessments, quality of life index, and tolerance were assessed. Adverse events were recorded. Daily application of the new treatment was well tolerated, and adverse events were absent. After 14 days, TEWL showed a 36.7% significant decrease (p = 0.035). At the end of the 28-day treatment, the Desquamation Index showed a reduction in 70% of patients; Eczema Area and Severity Index were reduced by 70.4% (p = 0.002); and skin irritation showed a significant reduction (p = 0.024). Likewise, Patient and Investigator Global Assessments reported a significant improvement in conditions and an overall global worsening when patients restarted their normal treatment. Parent's Index of Quality of Life Index significantly increased by 36.4% (p < 0.05) with Dermacare. In conclusion, a regular use of this new formulation can reduce the risk of relapse and extend the steroid-free treatment periods.

Background: Regesmohs registry is a nationwide registry including patients evaluated for Mohs surgery in 17 Spanish centres since July 2013. Given that Mohs surgery is the therapy with best results for high risk basal cell carcinoma (BCC) and other skin tumours, we wanted to describe the reasons that lead to some patients being excluded from this therapy and the alternative treatments that they received. These data may be useful to avoid excluding patients for Mohs surgery use, to estimate the healthcare demand of these patients and the demand for Hedgehog inhibitors therapy in this group. Objective: To describe patients excluded for Mohs surgery after pre-surgical assessment, and the treatments that they received. Methods: Regesmohs includes all consecutive patients assessed for Mohs surgery in the participating centres, collecting data on patient characteristics, intervention, and short and long-term results. Patients excluded for Mohs surgery after pre-surgical evaluation were described. Results: 3011 patients were included in Regesmohs from July 2013 to October 2016. In 85, Mohs surgery was not performed as they were considered inadequate candidates. 67 had BCC. Reasons for exclusion were: medical contraindication (27.1%, n=23) low-risk tumour in (18.8%, n=16) and giant tumour and bone invasion (15.3%, n=13). Only 1 patient (1.2%) showed lymph node involvement and no patients had visceral metastases. Of the 85 excluded patients, 29 (34.1%) were treated with conventional surgery, 24 (28.3%) with radiotherapy, 4 (4.7%) with inhibitors of the Hedgehog pathway (only indicated for BCC), and 2 (2.4%) received palliative care. We had no follow-up data on 14 patients (16.5%). CONCLUSION: Medical comorbidities were the most common reason for withholding Mohs surgery. Withholding therapy on the basis of distant extension is uncommon. Most excluded patients received simpler therapies: conventional surgery or radiotherapy, with hedgehog inhibitors being a new option. Copyright (C) 2017 AEDV. Published by Elsevier Espana, S.L.U. All rights reserved.

BackgroundThe two main tumors treated with Mohs micrographic surgery (MMS) are basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). There are no studies analyzing whether MMS is different when treating these two types of tumors. ObjectiveWe aim to compare the characteristics of the patients, the tumors, and MMS, and first-year follow-up of MMS in BCC and SCC. MethodsREGESMOHS is a prospective cohort study of patients treated with MMS. The participating centers are 19 Spanish hospitals where at least one MMS is performed per week. Data on characteristics of the patients, tumors, and surgery were recorded. The follow-up was done with two visits: the first visit within 1 month after surgery and the second one within the first year. ResultsFrom July 2013 to April 2017, a total of 2,669 patients who underwent MMS were included in the registry. Of them, 2,448 (93%) were diagnosed with BCC, and 181 (7%) were diagnosed with SCC. Patients with SCC were older than those with BCC (median age 73 years vs. 68 years) and presented immunosuppression more frequently. The tumor size was significantly larger in the SCC group. Regarding surgery, deeper invasion was more frequent in SCC, resulting in larger defects. Despite this, SCC did not require more stages to get clear margins or more time in the operating room. Incomplete Mohs was more frequent in the SCC group (6%) than in the BCC group (2%). The incidence of perioperative complications was higher when treating SCC. There were more relapses in the first-year follow-up in the SCC group. ConclusionThere are significant differences when comparing MMS in BCC and SCC. Knowledge of these differences can help to prepare the patient and plan the surgery, optimizing results.

Recent publication of the results of clinical trials in which lymph node dissection was not associated with any survival benefit in patients with sentinel node metastasis makes it necessary to reconsider the treatment of patients with melanoma. This article provides an update on the available evidence on the diverse factors (routes of metastatic spread, predictors, adjuvant therapy, etc.) that must be considered when treating patients with sentinel node-positive melanoma. The authors propose a decision-making algorithm for use in this clinical setting. The current evidence no longer supports lymph node dissection in patients with Low-risk sentinel node metastasis (sentinel node tumor load <= 1 mm). (C) 2018 AEDV. Published by Elsevier Espana, S.L.U. All rights reserved.

Background: The elderly population is increasing and more patients in this group undergo Mohs micrographic surgery (MMS). The few publications investigating MMS in elderly people conclude that it is a safe procedure; however, these are single-centre studies without a comparison group. Objective: To compare the characteristics of patients, tumours, MMS and 1-year follow-up in patients younger than 80 years, with patients older than 80 years at the time of surgery. Methods: Data was analysed from REGESMOHS, a prospective cohort study of patients treated with MMS. The participating centres were 19 Spanish hospitals where at least one MMS is performed per week. Data on characteristics of the patient, tumour and surgery were recorded. Follow-up data were collected from two visits; the first within 1 month postsurgery and the second within the first year. Results: From July 2013 to October 2016, 2575 patients that underwent MMS were included in the registry. Of them, 1942 (75.4%) were aged <80 years and 633 (24.6%) were ¿80 years old. In the elderly, the tumour size was significantly higher with a higher proportion of squamous cell carcinoma. Regarding surgery, elderly more commonly had tumours with deeper invasion and required a higher number of Mohs surgery stages, leaving larger defects and requiring more time in the operating room. Despite this, the incidence of postoperative complications was the same in both groups (7%) and there were no significant differences in proportion of relapses in the first-year follow-up. Conclusion: The risk of short-term complications and relapses were similar in elderly and younger groups. MMS is a safe procedure in the elderly.

Introduction: The Spanish Mohs Surgery Registry is used to collect data on the use and outcomes of Mohs micrographic surgery (MMS) in Spain. The aim of this study was to describe perioperative and intraoperative data recorded for MMS procedures performed between July 2013 (when the registry started) and January 2016. Material and methods: Prospective cohort study of data from 18 hospitals. The data collected included type of anesthesia, surgical technique, hospital admission, number of Mohs stages, management of preoperative risk factors, additional treatments, previous treatments, type of tumor, operating time, and complications. Results: Data were available for 1796 operations. The most common tumor treated by MMS was basal cell carcinoma (85.96%), followed by squamous cell carcinoma (6.18%), lentigo maligna (2.81%), and dermatofibrosarcoma protuberans (1.97%). Primary tumors accounted for 66.9% of all tumors operated on; 19.2% of tumors were recurrent and 13.9% were persistent. The most common previous treatment was surgical. MMS was mostly performed under local anesthesia (86.7% of cases) and as an outpatient procedure (71.8%). The frozen section technique was used in 89.5% of cases. One stage was needed to achieve tumor-free margins in 56.45% of patients; 2 stages were required in 32.1% of patients, 3 in 7.1%%, 4 in 2.7%, and 5 or more in 1.8%. The defect was reconstructed by the dermatologist in 98% of patients and the most common technique was flap closure (47.2%). Intraoperative complications were recorded for just 1.62% of patients and the median (interquartile range) duration of surgery was 75 (60-100) minutes. Conclusion: The characteristics of the patients and tumors treated by MMS are similar to those reported for similar studies in other geographic areas. Lentigo matigna and dermatofibrosarcoma protuberans accounted fora higher proportion of cases in our series, and repair of the surgical defect by a dermatologist was also more common. Operating times in MMS are not much longer than those reported for other procedures and the rate of intraoperative complications is very low. (C) 2017 AEDV. Published by Elsevier Espana, S.L.U. All rights reserved.

BACKGROUND: The typical reconstructive option for closing large-sized defects of the distal half of the nose is the paramedian forehead flap. Other alternatives are a melolabial interpolation flap and bilobed or trilobed flaps. The dorsal nasal (Rieger) flap is suitable for closing small-sized defects at this location, especially when they are medially located. OBJECTIVE: The authors describe a modified dorsal nasal flap reconstruction for large nasal defects. The novelty of this study lies in lengthening the leading edge of flap rotation, which may provide tissue either from the adjacent nasal skin, the nasofacial groove, or the cheek. MATERIALS AND METHODS: The authors performed a retrospective chart review of all patients with large defects (>20 mm) of the nose who underwent modified dorsal nasal flap repair between January 2004 and March 2015 at a single academic center. RESULTS: Twenty-seven patients (16 male, 11 female; ages 44-88, mean age 62 years) had defects (the smallest 15 × 21 mm, and the largest 32 × 37 mm) on the lower portion of the nasal pyramid. Follow-up ranged from 12 months to 11 years with good or excellent results in all cases. CONCLUSION: Elongated dorsal nasal flap is a reproducible one-stage flap for large defects of the nose, with minimal risk of aesthetic or functional complications.

Although capillary malformations (CMs) are not usually serious health problems in themselves, they can occasionally be warning signs for syndromes with more serious or aggressive vascular malformations not readily apparent at birth or on initial examination. We describe a series of 9 patients with a common phenotype: (1) CM on the fingertips; (2) associated combined vascular (lymphatic-venous) malformations on the trunk and/or extremities; and (3) in some cases, partial overgrowth and asymmetry of the extremities. Data were collected retrospectively for patients with CM on the fingertips who were treated at 2 Vascular Anomalies Centers from January 2006 to January 2016. All the patients presented CM and other associated vascular abnormalities from birth. CMs were centripetal in their distribution. The greater the centripetal progression of the CM, the greater the associated vascular malformation observed. Some patients also presented varying degrees of overgrowth and asymmetry. At present, we have not found a patient with CM of the fingertip without associated anomalies. In conclusion, we present a series of patients with a particular morphology of CM located on the fingertips. All cases were associated with lymphatic-venous malformations at other sites. These data lead us to recommend these patients be referred from birth to a multidisciplinary unit of vascular anomalies.

Introduction: The basic principle of a lobed or finger-like transposition flap is that, after covering the defect with the transposed tissue, the donor site is closed primarily. With large defects, a second lobe may be added to the flap if primary closure of the area left by the first lobe is not possible. The flap can often be made to adapt to the defect, but this maneuver, in combination with primary closure of the adjacent tissue, can sometimes produce excessive tension and compromise the blood supply. Material and methods: We present a series of 4 patients with epithelial tumors of the lateral wall of the nose. The defects left by surgical excision were covered by finger-like transposition flaps. Subcutaneous sutures called guitar-string sutures were used to reduce the size of the defect and facilitate tension-free closure. Conclusions: We propose use of the guitar-string subcutaneous suture in those cases in which the defect is larger than the area that can be covered by the flap. This will make it easier to adapt the flap to the defect and will reduce the risk of excessive tension causing necrosis of the transposed tissue. (C) 2017 Published by Elsevier Espana, S.L.U. on behalf of AEDV.

Background Infantile hemangiomas with minimal or arrested growth (IH-MAGs) are characterized by a proliferative component of <25% of its surface area. The co-occurrence of IH-MAGs and soft tissue anomalies is rare, and case series of this association are lacking. Objective We present 10 cases of IH-MAGs associated with soft tissue hypertrophy and describe their clinical features. Methods We reviewed all infantile hemangiomas with minimal or arrested growth seen between 2009 and 2016 in the dermatology clinic department at Hospital Santa Creu i Sant Pau, Barcelona. To collect more patients, we also requested cases from the Hemangioma Investigator Group and members of the Spanish Society of Vascular Anomalies. Results Ten patients had IH-MAGs associated with soft tissue hypertrophy; seven involving the arm and three involving the leg. All displayed a segmental pattern, a doughy and puffy texture and prominent surface veins. No significant asymmetries in limbs and no other visceral anomalies were observed at follow-up (range 15 months to 7 years). One patient reported coldness in the limb with infantile hemangioma, but RMI-angiography did not disclose a vascular malformation underneath the lesion. Ulceration was observed in three patients. The proliferative component in all IH-MAGs had faded at 1-year follow-up, while soft tissue hypertrophy and prominent vessels remained unchanged. Conclusions In this first case series of IH-MAGS associated with soft tissue hypertrophy, soft tissue hypertrophy was not progressive and remained unchanged over time, unlike the proliferative component of classic infantile hemangioma. The origin of the prominent vessels and the higher ulceration risk are unknown; however, these findings are probably related to a minor disruption of local vessels not detected in imaging tests.

Introducción El principio básico de un colgajo lobulado o digitiforme de trasposición es que una vez que el tejido desplazado cubra el defecto, la zona dadora cierre directamente. Cuando los defectos son grandes puede ser necesaria la realización de un segundo lóbulo, debido a que el área que deja el primer lóbulo con su movimiento no cumple el criterio anterior. Con frecuencia se puede forzar el colgajo y adaptarlo al nuevo lecho, aunque a veces esta maniobra, sumada al cierre directo del tejido adyacente, puede traccionar en exceso y comprometer la vascularización. Material y métodos Se presenta una serie de 4 pacientes con tumores epiteliales en el lateral nasal. Tras la extirpación quirúrgica, los defectos resultantes se cubrieron mediante colgajos digitiformes de trasposición. En el diseño del cierre de los defectos se utilizaron unos puntos de sutura subcutáneos denominados en «cuerda de guitarra» para disminuir el tamaño del área cruenta y facilitar el ensamblaje del colgajo sin tensión. Conclusiones Proponemos la realización de la sutura subcutánea en «cuerda de guitarra» para aquellos casos en los que el defecto cutáneo es mayor que la cobertura que aporta el colgajo local, con el objetivo de facilitar su ensamblaje y disminuir el riesgo de necrosis del tejido desplazado por una excesiva tensión.

Actinic keratosis is a precursor lesion to the most common nonmelanoma skin cancer. Conventional photodynamic therapy (PDT) has been shown to be effective, but the procedure is time-consuming, can be very painful, and requires infrastructure. These shortcomings led to the emergence of daylight PDT. To obtain a global estimate of efficacy, we undertook a systematic literature review and performed a meta-analysis of the available evidence on the efficacy and safety of daylight PDT as compared to conventional PDT in the treatment of actinic keratosis and/or field cancerization. The conclusion is that the difference in efficacy is clinically negligible (global estimate of the mean response rate difference, ¿3.69%; 95% CI, ¿6.54% to ¿0.84%). The adverse effects of daylight PDT are mild and localized (79% of patients report no discomfort), and patients report less pain (P < .001). Daylight PDT gives good to excellent cosmetic results in more than 90% of patients, and patient satisfaction is greater (P < .001).

Introducción Los hemangiomas infantiles son tumores benignos producidos por la proliferación de células endoteliales de vasos sanguíneos, con una alta incidencia en niños menores de un año (4-10%) y se estima que un 12% de ellos requiere tratamiento. Dicho tratamiento debe realizarse según las guías de práctica clínica y la experiencia de los especialistas, las características de los pacientes y las preferencias de sus progenitores. Métodos El proceso de consenso se realizó utilizando evidencias científicas sobre el diagnóstico y tratamiento de los hemangiomas infantiles, extraídas mediante una revisión sistemática de la literatura, junto con el juicio experto de los especialistas. Las recomendaciones formuladas fueron validadas por los especialistas, aportando su grado de acuerdo. Resultados El presente documento recoge recomendaciones sobre la clasificación, las asociaciones, las complicaciones, el diagnóstico, el tratamiento y el seguimiento de los pacientes con hemangioma infantil. Además, se incluyen algoritmos de actuación y se aborda el manejo multidisciplinario y criterios de derivación entre los distintos especialistas que participan en el manejo clínico de este tipo de pacientes. Conclusiones Las recomendaciones y los algoritmos diagnóstico y terapéutico de los hemangiomas infantiles recogidos en este documento son una herramienta útil en el manejo adecuado de estos pacientes.

The logarithmic spiral flap is useful for the closure of circular or oval defects situated on the lateral surface of the nose and nasal ala. The flap initiates at one of the borders of the wound as a pedicle with a radius that increases progressively to create a spiral. We propose the logarithmic spiral flap as an excellent option for the closure of circular or oval defects of the nose

The logarithmic spiral flap is useful for the closure of circular or oval defects situated on the lateral surface of the nose and nasal ala. The flap initiates at one of the borders of the wound as a pedicle with a radius that increases progressively to create a spiral. We propose the logarithmic spiral flap as an excellent option for the closure of circular or oval defects of the nose.

Background Unmet needs exist in actinic keratosis (AK) treatment. Daylight photodynamic therapy (DL-PDT) has shown good efficacy and safety results compared to conventional PDT (c-PDT) in a recent Phase III multi-centre randomised controlled trial in Australia among 100 subjects with AKs. Objectives Demonstrate non-inferior efficacy and superior safety of DL-PDT compared to c-PDT in treating multiple mild and/or moderate facial/scalp AKs. Methods Phase III, 12 week, multi-centre, randomised, investigator-blinded, controlled, intra-individual study conducted at different latitudes in Europe. AKs of adult subjects were treated once with methyl aminolevulinate (MAL) DL-PDT on one side of the face and MAL c-PDT contralaterally. Endpoints for DL-PDT concerned efficacy (non-inferiority regarding complete lesion response at week 12) and safety (superiority regarding subject's assessment of pain after treatment, on an 11-point numeric rating scale). Safety evaluation also included incidence of adverse events. Subject satisfaction was described using a questionnaire at baseline and last visit. Results At week 12, the total lesion complete response rate with DL-PDT was similar (non-inferior) to c-PDT (70% vs. 74%, respectively; 95% CI [¿9.5; 2.4] in PP analysis, confirmed in ITT analysis). In addition, efficacy of DL-PDT was demonstrated regardless of weather conditions (sunny or cloudy). DL-PDT was nearly painless compared to c-PDT (0.7 vs. 4.4, respectively; P < 0.001), better tolerated and resulted in higher subject satisfaction. Conclusion DL-PDT in comparison with c-PDT was as effective, better tolerated and nearly painless with high patient satisfaction, and may be considered a treatment of choice to meet needs of patients with mild or moderate facial/scalp AKs.

Introduction The Spanish registry of Mohs micrographic surgery started collecting data in July 2013. The aim of the registry is to report on the use of this technique in Spain and the outcomes achieved. In the present article, we describe the characteristics of patients and the tumors treated. Material and methods This is a prospective cohort study of patients treated with Mohs micrographic surgery. The participating centers are hospitals where at least one intervention of this type is performed each week. All patients considered for Mohs micrographic surgery in participating centers are included in the registry except those who have been declared legally incompetent. Results Between July 2013 and October 2014, data from 655 patients were included in the registry. The most common tumor involved was basal cell carcinoma, and the most common histological subtype was infiltrative basal cell carcinoma. Most of the tumors treated were located on the face or scalp, and the most common site was the nose. Almost 40% of the tumors treated were recurrent or persistent, and preoperative tumor size was similar to that reported in other European studies and in Australia. In total, 45.5% of patients had received previous surgical treatment. Conclusion The findings are similar to those reported in other studies, and the data collected are useful for assessing whether the results of studies carried out elsewhere are applicable in Spain.

An 82-year-old man presented with a 1-year history of swelling of his left ear, with associated difficulty hearing. He had applied topical glucocorticoids for 4 months without response. On examination, the ear was enlarged, indurated, and erythematous (Panel A), with no associated lymphadenopathy. Biopsy revealed chronic inflammatory infiltration in the dermis, with confluent epithelioid granulomas and a lymphocytic background. High magnification showed abundant intracytoplasmic amastigotes in dermal macrophages (Panel B). Leishmania infantum was identified by means of culture and polymerase-chain-reaction assay. No visceral enlargement was noted on ultrasonography of the abdomen. The patient was treated with amphotericin B, with complete resolution of the lesion after 2 months (Panel C) and recovery of hearing loss.

Context: Hyaluronic acid (HA) is the most common filler used to rejuvenate. Today, a three-dimensional approach prevails over previous techniques in which this material was used in specific areas of the face such as the nasolabial fold, the marionette line, and the eye trough giving a strange appearance that does not look natural. Even with a volumizing purpose, the injection of HA can sometimes produce clinically detectable nodules or lumps where the filler is deposited. Aims: To develop a new technique of injecting HA that can provide more natural results and avoid the lumpiness and nodular appearance that sometimes occurs with the injection of HA. To detect whether mixing HA with diluted anesthetic agent modifies its behavior. Settings and Design: Prospective, case control, single-center study on a private clinic setting. Materials and Methods: Eighty six patients were enrolled in this study. All of them had a previous treatment with nondiluted HA using a needle at least a year before. Patients were injected with 8 mL of reticulated HA (RHA) mixed with 6 mL of saline and 2 mL of anesthetic agent. The mixture was administered through a cannula inserted in the face, one at mid-cheek and another at frontal-temporal point of entry. Owing to the lifting effect of this mixture we called this procedure liquid lift (LL). Patients were evaluated 1 month, 6 months, and a year later and asked to compare the LL with previous experiences in terms of natural look, pain, and appearance of nodules. Statistical Analysis Used: Student's t-test. Results: One month after the treatment, 83 out of 86 patients (96.5%) thought LL produced a more natural look than the previous treatment with the needle. Sixty two (72%) considered LL less painful than the previous treatment and only eight (9.3%) could detect lumps or nodules 1 month after LL was performed compared with 46 (53.5%) that described this problem with previous needle injections. The incidence of bruising was also clearly lower (7% with LL vs 17.4% with traditional needle). Conclusions: Injection of diluted HA with saline and anesthetic agents through a cannula all over the face or LL can provide more natural results and less lumps or nodules, and is less painful than traditional treatments involving needle injection of nondiluted HA.

Background Sturge-Weber syndrome (SWS) is characterized by port-wine stains (PWS) affecting the face, eyes, and central nervous system. Pulsed dye laser (PDL) is the standard treatment for PWS. Unfortunately, recurrence is frequent because of reformation and reperfusion of blood vessels. Objective We sought to assess the clinical efficacy of topical rapamycin combined with PDL in PWS of patients with SWS. Methods We conducted a phase II, randomized, double-blind, intraindividual placebo-controlled, clinical trial. We recruited 23 patients with SWS and facial PWS (12 women; median age 33 years, age range 17-65 years) from the University Clinic of Navarra, Spain. Four interventions were evaluated: placebo, PDL + placebo, rapamycin, and PDL + rapamycin. Clinical and histologic responses were evaluated using a chromatographic computerized system, spectrometry, and histologic analyses at 6, 12, and 18 weeks after the intervention. Results PDL + rapamycin yielded the lowest digital photographic image score and the lowest percentage of vessels in histologic analysis, and showed a statistically significant improvement compared with the other interventions. The treatment was generally well tolerated. Limitations PDL was only applied to the lateral parts of the PWS area. Conclusion Topical rapamycin associated with PDL seems to be an effective treatment for PWS in patients with SWS.

Venous leg ulcers (VLU) generally have slow healing rates (HR) and frequent recurrence rates (RR). The underlying etiology is venous hypertension. The present observational cohort study was to determine healing and RR in VLU treated with ultrasound-guided foam sclerotherapy (UGFS). One hundred and eighty VLU were treated with polidocanol microfoam monthly under ultrasound control. Median follow-up was 30 months (range: 17-40). One hundred seventy-two (95.6%) ulcers healed during the study. The overall twenty-four week HR was 79.4% and was significantly higher (95.1%) in patients with isolated great saphenous vein incompetence than in those with great saphenous vein plus perforator (91.7%) or exclusive perforator incompetence (78.9%) (p¿<¿0.01). Patients without deep vein incompetence had a significantly higher 6-month HR (89.8%) than those with (67.4%) (p¿<¿0.01). Multivariate analysis identified the following independent risk factors: chronicity¿>¿12 months (OR 7.69), area¿>¿6¿cm(2) (OR 4.24), lipodermatoesclerosis (OR 12.22), history of¿>¿3 previous ulcers (OR 5.57) and history of deep vein thrombosis (OR 6.18). One, two and three year ulcer RR were 8.1%, 14.9%, and 20.8%, respectively. Isolated perforator incompetence and previous history of venous surgery were significantly (p¿=¿0.03) associated with a higher RR. VLU treated with microfoam sclerotherapy are associated with high HR and low mid-term RR.

Las características de nuestro entorno sugieren que enfermedades como la queratosis actínica (QA) aumentarán su prevalencia y, en consecuencia, la demanda asistencial en los próximos años. Deben tenerse en cuenta una extensa lista de características clínicas en el abordaje terapéutico de la QA, hasta hace poco compuesto únicamente por técnicas ablativas y exclusivamente dirigidas a las lesiones, sin considerar el campo de cancerización. El incremento del arsenal terapéutico de los últimos años hace necesaria la homogenización de criterios que faciliten la elección de la mejor opción para cada paciente. La formulación de recomendaciones de consenso entre expertos a partir de la revisión de las evidencias científicas en cuanto a diagnóstico y tratamiento disponibles, permite aportar conocimiento dirigido a la mayor calidad en la atención de los pacientes, facilita una mayor homogeneidad en la toma de decisiones y promueve la sensibilización necesaria de todos los agentes sanitarios involucrados.

Background and objectives The Spanish National Cutaneous Melanoma Registry (Registro Nacional de Melanoma Cutáneo [RNMC]) was created in 1997 to record the characteristics of melanoma at diagnosis. In this article, we describe the characteristics of these tumors at diagnosis. Patients and methods This was a cross-sectional observational study of prevalent and incident cases of melanoma for which initial biopsy results were available in the population-based RNMC. Results The RNMC contains information on 14,039 patients. We analyzed the characteristics of 13,628 melanomas diagnosed between 1997 and 2011. In total, 56.5% of the patients studied were women and 43.5% were men. The mean age of the group was 57 years (95% CI, 56.4-57 years) while median age was 58 years. The most common tumor site was the trunk (37.1%), followed by the lower limbs (27.3%). The most frequent clinical-pathologic subtype was superficial spreading melanoma (n=7481, 62.6%), followed by nodular melanoma (n=2014, 16.8%). Localized disease was observed in 86.2% of cases (n=10,382), regional metastasis in 9.9% (n=1188), and distant metastasis in 3.9% (n=479). Independently of age at diagnosis, men had thicker tumors, more ulceration, higher lactate dehydrogenase levels, and a higher rate of metastasis than women (P<.001). Conclusions Based on our findings, melanoma prevention campaigns should primarily target men over 50 years old because they tend to develop thicker tumors and therefore have a worse prognosis.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant skin tumor that may also infiltrate dermis and subcutaneous tissue. Although the mainstay of treatment has been wide local excision, during the last decade three-dimensional (3D) histology surgery has proven very effective for the treatment of this tumor. OBJECTIVE: We sought to evaluate the effectiveness of 3D histology surgery for the treatment of DFSP. METHODS: We retrospectively reviewed charts of patients with DFSP treated in our unit with 3D histology surgery between April 2000 and May 2011. Age at onset, gender, duration of tumor, previous treatment, lesion site, number of surgical stages, postsurgical defect size, closure technique, and follow-up were registered. RESULTS: A total of 29 patients were included. Mean patient age was 40.7 years. Fifteen lesions were located on the trunk, 11 on the extremities, and 3 in the head and neck region. Twelve patients had primary tumors and 17 were referred to us after incomplete excision. The average number of 3D histology surgical stages required for tumor clearance was 1.4. Mean postsurgical defect size was 26 cm(2). All lateral and deep borders excised were tumor-free. Mean follow-up period was 68 months (range 12-142 months) with a 0% recurrence rate. LIMITATIONS: This was a retrospective review. CONCLUSION: Three-dimensional histology surgery with paraffin sections is effective for the treatment of DFSP and feasible in an outpatient setting. The low recurrence rates obtained confirm the oncologic efficacy of the procedure. Furthermore, designing closure on the basis of focally affected margins improves functional and aesthetic outcomes without compromising oncological effectiveness.

Patients were followed for up to 6 months using clinical assessment of achromic lesions. Treated areas ranged between 4¿cm(2) and 210.6¿cm(2). Response to treatment was excellent in all patients with 90-95% repigmentation success rate.

There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformation

At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel¿Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenit

: The presence of pulmonary arterial hypertension in patients with extensive slow-flow vascular malformations is not an isolated feature but is relatively frequent. Levels of D-dimer correlate with PASP in these patients.