Risk of Second Primary Malignancies in Melanoma Survivors: A Population-Based Study.

(1) Introduction: The association between melanoma (MM) and the occurrence of second primary neoplasms (SPNs) has been extensively studied, with reported incidence rates ranging from 1.5% to 20%. This study aims to evaluate the occurrence of SPNs in patients with a history of primary MM and to describe the factors that make the risk higher in our population. (2) Material and Methods: We conducted a prospective cohort study and calculated the incidence rates and relative risks (RR) for the development of different SPNs in 529 MM survivors from 1 January 2005 to 1 August 2021. Survival and mortality rates were obtained, and the Cox proportional hazards model was used to determine the demographic and MM-related factors that influence the overall risk. (3) Results: Among the 529 patients included, 89 were diagnosed with SPNs (29 prior to MM diagnosis, 11 synchronous, and 49 after MM), resulting in 62 skin tumors and 37 solid organ tumors. The estimated probability of developing SPNs after MM diagnosis was 4.1% at 1 year, 11% at 5 years, and 19% at 10 years. Older age, primary MM location on the face or neck, and histologic subtype of lentigo maligna mm were significantly associated with a higher risk of SPNs. (4) Conclusions: In our population, the risk of developing SPNs was higher in patients with primary MM located on the face and neck and with the histological subtype of lentigo maligna-MM. Age also independently influences the risk. Understanding these hazard factors can aid in the development of MM guidelines with specific follow-up recommendations for individuals with the highest risk.

Inter- and intra-observer variability in the selection of therapy for infantile hemangiomas among pediatric dermatologists in Spain.

BACKGROUND: Guidelines and expert recommendations on infantile hemangiomas (IH) are aimed at increasing homogeneity in clinical decisions based on the risk of sequelae. OBJECTIVE: The objective was to analyze the inter- and intra-observer agreement among pediatric dermatologists in the choice of treatment for IH. METHODS: We performed a cross-sectional inter-rater and intra-rater agreement study within the Spanish infantile hemangioma registry. Twenty-seven pediatric dermatologists were invited to participate in a survey with 50 clinical vignettes randomly selected within the registry. Each vignette contained a picture of an infantile hemangioma with a clinical description. Raters chose therapy among observation, topical timolol, or oral propranolol. The same survey reordered was completed 1 month later to assess intra-rater agreement. Vignettes were stratified into hemangioma risk categories following the Spanish consensus on IH. The agreement was measured using kappa statistics appropriate for the type of data (Gwet's AC1 coefficient and Gwet's paired t test). RESULTS: Twenty-four dermatologists completed the survey. Vignettes represented 7.8% of the Spanish hemangioma registry. The inter-rater agreement on the treatment decision was fair (AC1  = 0.39, 95% confidence interval [CI]: 0.30-0.47). When stratified by risk category, good agreement was reached for high-risk hemangiomas (AC1  = 0.77, 95% CI: 0.51-1.00), whereas for intermediate- and low-risk categories, the agreement was only fair (AC1 0.31, 95% CI: 0.16-0.46 and AC1  = 0.38, 95% CI: 0.27-0.48, respectively). Propranolol was the main option for high-risk hemangiomas (86.4%), timolol for intermediate-risk (36.8%), and observation for low-risk ones (55.9%). The intra-rater agreement was good. The inter-rater agreement between pediatric dermatologists on the treatment of IH is only fair. Variability was most significant with intermediate- and low-risk hemangiomas.

Síndrome de epidermólisis ampollosa juntural-atresia pilórica con aplasia cutis congénita extensa / Junctional epidermolysis bullosa-pyloric atresia syndrome with extensive congenital aplasia cutis

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[Effects of solar radiation and an update on photoprotection]. / Efectos de la radiación solar y actualización en fotoprotección.

Sunburn, immunodepression, photoaging, and photocarcinogenesis, are some of the most significant adverse effects of solar radiation in humans. Children are population group of special vulnerability, due to the fact that exposure to the sun has more pronounced biological effects compared to adults. Furthermore, childhood is a critical period for promoting the development of photo damage and photocarcinogenesis in the later stages of life if adequate measures at not put into place. This is because it is estimated that between 18 and 20 years of age is when 40% to 50% of the accumulative exposure to ultraviolet radiation up to 60 years of age is received. The most important strategy for the photoprotection of children is changes in behaviour and habits associated with exposure to the sun at all levels (school, society, family, etc.). Resorting to the shade, reduction in overall time of exposure to the sun, and physical protection (clothes, hats, and sunglasses) are the best and least costly photoprotection strategies. The photoprotectors must be incorporated into the daily routine of children in the same way as adults, and must complete a series of requirements in order to make them effective, safe, and in line with the environment.

Efectos de la radiación solar y actualización en fotoprotección / Effects of solar radiation and an update on photoprotection

Las quemaduras solares, la inmunodepresión, el fotoenvejecimiento y la fotocarcinogénesis son algunos de los efectos adversos más importantes de la radiación solar en el ser humano. Los niños son un grupo poblacional de especial vulnerabilidad debido a que en ellos la exposición solar tiene efectos biológicos más pronunciados en comparación con los adultos. Por otro lado, la infancia es un periodo crítico para promover el desarrollo de fotodaño y fotocarcinogénesis en etapas más tardías de la vida si no se ponen las medidas adecuadas, ya que se estima que entre los 18 y 20 años de edad se recibe del 40-50% de la exposición acumulativa a la radiación ultravioleta hasta la edad de 60 años. La estrategia más importante para la fotoprotección de los niños son las modificaciones de comportamiento y hábitos relacionados con la exposición al sol a todos los niveles (colegio, sociedad, familia, etc.). El recurso de la sombra, la reducción del tiempo global de exposición al sol y la protección física (ropa, sombreros y gafas de sol) representan las mejores y menos costosas estrategias de fotoprotección. Los fotoprotectores deben incorporarse a la rutina diaria de los niños, de la misma manera que los adultos, y deben cumplir una serie de requisitos que los hagan eficaces, seguros y comprometidos con el medio ambiente

Sunburn, immunodepression, photoaging, and photocarcinogenesis, are some of the most significant adverse effects of solar radiation in humans. Children are population group of special vulnerability, due to the fact that exposure to the sun has more pronounced biological effects compared to adults. Furthermore, childhood is a critical period for promoting the development of photo damage and photocarcinogenesis in the later stages of life if adequate measures at not put into place. This is because it is estimated that between 18 and 20 years of age is when 40% to 50% of the accumulative exposure to ultraviolet radiation up to 60 years of age is received. The most important strategy for the photoprotection of children is changes in behaviour and habits associated with exposure to the sun at all levels (school, society, family, etc.). Resorting to the shade, reduction in overall time of exposure to the sun, and physical protection (clothes, hats, and sunglasses) are the best and least costly photoprotection strategies. The photoprotectors must be incorporated into the daily routine of children in the same way as adults, and must complete a series of requirements in order to make them effective, safe, and in line with the environment

Congenital tufted angioma: A multicenter retrospective study of 30 cases.

BACKGROUND: Recent reports indicate that tufted angioma is a rare vascular neoplasm that manifests more frequently at birth than previously thought. Few studies specifically address congenital presentation. OBJECTIVES: We analyzed the clinicopathological characteristics, clinical course, and treatment of congenital tufted angioma (cTA) and evaluated variables that were indicative of problematic lesions. METHODS: We performed an observational retrospective study of 30 patients with cTA in 9 Spanish hospitals over a 14-year period. Histopathology and immunohistochemistry studies were performed. RESULTS: Congenital tufted angioma mainly affected the limbs (56.67%), followed by the face and/or neck (23.33%). Almost three-quarters of facial cTA were located over the mandibular area. Immunohistochemically, proliferating cells expressed markers of endothelial cells, with some clusters of cells, especially at the periphery of the aggregates, showing positivity for podoplanin. As no associated complications were observed in 66.67% of cases, no treatment was started. LIMITATIONS: Data were collected retrospectively. CONCLUSIONS: Our findings emphasize the clinical features and course of cTA. The possibility of cTA should be considered when a poorly defined congenital infiltrative vascular tumor with(out) overlying hirsutism appears over the mandibular area. Location on the face and/or neck requires a more comprehensive workup, since potentially severe complications often appear early.

Alemtuzumab in refractory Sézary syndrome.

Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by the triad of erythroderma, lymphadenopathy and circulating atypical cells. The emergence of new molecular targets has enabled the development of drugs such as alemtuzumab, an anti-CD52 monoclonal antibody, which has shown promising results in the treatment of this entity. We report the case of a 70-year-old male with refractory Sézary syndrome in whom treatment with alemtuzumab achieved an 80% skin lesion clearance with complete haematologic and radiologic response. The treatment was discontinued after 4 months due to adverse effects, with the patient showing a sustained response without disease progression after 13 months of follow-up.

Alemtuzumab in refractory Sézary syndrome

Abstract: Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by the triad of erythroderma, lymphadenopathy and circulating atypical cells. The emergence of new molecular targets has enabled the development of drugs such as alemtuzumab, an anti-CD52 monoclonal antibody, which has shown promising results in the treatment of this entity. We report the case of a 70-year-old male with refractory Sézary syndrome in whom treatment with alemtuzumab achieved an 80% skin lesion clearance with complete haematologic and radiologic response. The treatment was discontinued after 4 months due to adverse effects, with the patient showing a sustained response without disease progression after 13 months of follow-up.

Otophyma: a rare variant of phymatous rosacea.

BACKGROUND: Phymatous rosacea is characterized by thickened skin and irregular surface contours as the result of variable combinations of fibrosis, sebaceous hyperplasia and lymphoedema. Otic phyma is rarely seen and has been rarely reported in the English literature. METHODS: We present another case of this uncommon condition, frequently misdiagnosed, maybe due to its underrecognition. RESULTS: Phymatous rosacea most commonly occurs on the nose, but may also develop on any sebaceous facial region, including the ears. Although it is a benign condition, there are significant morbidities associated with rosacea, and can even result in conductive deafness because of the obstruction of the external auditory canal. CONCLUSIONS: The knowledge of this entity may be important for clinicians, especially dermatologists, ENT specialists and plastic surgeons, for an appropriate treatment and follow-up.

Subcutaneous phaeohyphomycosis due to Alternaria infectoria in a renal transplant patient: surgical treatment with no long-term relapse / Feohifomicosis subcutánea debida a Alternaria infectoria en un paciente sometido a un trasplante renal: tratamiento quirúrgico sin recidivas a largo plazo

Background. Phaeohyphomycosis can be caused by a number of different species, being the most common Alternaria alternata and Alternaria infectoria. The biggest risk factor for the development of the infection is immunosuppression. Aims. We present the case of a 64-year-old male renal transplant patient who came to hospital for presenting a tumour in the Achilles region which had been gradually growing in size. Methods. A skin biopsy was taken for histological study and culture of fungi and mycobacteria. Blood tests and imaging studies were performed. Results. Histopathology study and cultures identified A. infectoria as the causal agent. Imaging studies ruled out internal foci of infection. The lesion was surgically removed with no signs of recurrence after 24 months of follow-up. Conclusions. There are no treatment guidelines at present for cutaneous and subcutaneous Alternaria spp. infections. Various systemic antifungals have been used, either in combination with surgical removal or alone, with varying results. Surgery alone could be useful in the treatment of solitary, localised lesions in transplant patients in whom there are difficulties in controlling immunosuppression (AU)

Antecedentes. La feohifomicosis está causada por diversas especies de hongos, siendo las más habituales Alternaria alternata y Alternaria infectoria. El factor de riesgo principal en la aparición de la infección es la inmunosupresión. Objetivos. Presentamos el caso de un hombre de 64 años de edad, sometido a un trasplante renal, que se presentó en el hospital con un tumor en la región del tendón de Aquiles que había aumentado gradualmente de tamaño. Métodos. Se obtuvo una biopsia de piel para estudio histológico y cultivo de hongos y micobacterias. Se efectuaron análisis de sangre y estudios de diagnóstico por imagen. Resultados. El estudio histopatológico y los cultivos permitieron la identificación de A. infectoria como del patógeno causal. Los estudios por imagen descartaron focos internos de infección. Se procedió a la exéresis quirúrgica de la lesión sin signos de recidiva después de 24 meses de seguimiento. Conclusiones. En la actualidad no se dispone de guías de tratamiento para las infecciones cutáneas y subcutáneas por Alternaria spp. Se han utilizado diversos antimicóticos sistémicos, combinados con la exéresis quirúrgica o solos, con diferentes resultados desiguales. La cirugía sola podría ser útil en el tratamiento de las lesiones localizadas, solitarias, en los pacientes sometidos a un trasplante en los que es difícil controlar la inmunosupresión (AU)