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Relatively little is known about the true aggressive potential of pleomorphic dermal sarcoma (PDS) or optimal management strategies. To describe the outcomes of 16 cases of PDS treated at our hospital (14 with modified Mohs micrographic surgery [M-MMS] and two with conventional surgery) and establish an adequate plan for management. We reviewed 16 PDS cases treated at our hospital between October 2007 and June 2019 and compared our results with the available evidence. In total, 69% of cases had recurred after initial conventional surgery, M-MMS led to local disease control in 83% of cases, and 19% of patients developed metastasis. Combining all published PDS cases with ours, we calculated an overall metastasis rate of 12%, and an overall recurrence rate of 35% after conventional surgery and 17% after M-MMS. PDS is more aggressive than previously estimated, with an overall metastatic rate of 12%. Despite high recurrence rates with previous conventional surgery (69%), M-MMS achieved a good rate of local disease control (83%). Given the potential aggressivity of PDS and the importance of clear surgical margins, M-MMS appears to be more adequate than conventional excision. Staging studies and close monitoring are warranted in PDS patients, for which we propose a management algorithm.

Necrobiosis lipoidica is a rare chronic granulomatous disease. Multiple treatment approaches are available, but results are generally minimal and inconsistent. Some publications report variable results with photodynamic therapy (PDT) as a second line of treatment for refractory cases. We report 4 cases of necrobiosis lipoidica treated satisfactorily with conventional PDT using methyl aminolevulinate or 5-aminolevulinic acid BF-200 as the photosensitizing agent. All 4 patients were women with diabetes mellitus who had undergone treatment at least twice in the past, with little improvement. The lesions resolved completely with PDT, leaving only residual atrophy after a mean of 3.2 sessions per lesion.

Resumen La necrobiosis lipoidica (NL) es una enfermedad granulomatosa cronica poco frecuente para la que existen multitud de tratamientos disponibles. No obstante, estos ofrecen habitualmente minimos e inconsistentes resultados. En algunas publicaciones se describe el tratamiento con terapia fotodinamica (TFD) como tratamiento de segunda linea en casos refractarios, con resultados variables. Comunicamos 4 casos de NL tratados satisfactoriamente con TFD convencional con MAL y BF-200 ALA. Las 4 pacientes eran mujeres afectas de diabetes mellitus y todas habian recibido al menos 2 tratamientos previos con escaso resultado. Tras una media de 3,2 sesiones de TFD por lesion, las 4 pacientes presentaron una resolucion completa de las lesiones, persistiendo unicamente atrofia residual.

Background Dermatofibrosarcoma protuberans (DFSP) is an invasive skin tumor traditionally associated with very high recurrence rates when treated with conventional surgery (CS). Objective To calculate the minimum margin that would have been required to achieve complete tumor clearance with hypothetical CS. To analyze DFSP characteristics and Mohs micrographic surgery (MMS) effectiveness in treatment of this tumor. Materials and methods Minimum margin was calculated by measuring the largest distance from the visible edge of the tumor to the edge of the surgical defect. Tumor variables (age, sex, size, time since onset, and location) were correlated with surgical variables (number of stages and minimum margin). Results We studied 222 cases of DFSP treated with MMS. A mean of 1.47 MMS stages and a mean minimum margin of 1.23 cm were required to achieve tumor clearance. Tumors on the head and neck required significantly more stages and a significantly wider margin. Tumor size was positively correlated with time to diagnosis, age, and number of MMS stages. Conclusion Tumors located on the head and neck have greater subclinical extension. Tumor size was also a predictor of surgical difficulty, but time to diagnosis was not.

There are 3 types of leiomyosarcoma of the skin: dermal, subcutaneous, and metastatic cutaneous. Dermal leiomyosarcoma arises from smooth muscle fibers in arrector pili muscles, genital dartos muscles, and the nipple-areola complex. It is an intermediate-grade tumor associated with a tendency for local recurrence (24%) and low metastatic potential (4%). Subcutaneous leiomyosarcoma originates from smooth muscle in blood vessel walls and has higher rates of local recurrence (37%) and metastasis (43%). Plemorphic dermal sarcoma typically affects elderly patients and arises in sun-exposed areas (e.g., the scalp). Its histologic and immunohistochemical characteristics are similar to those of atypical fibroxanthoma, but it is more aggressive (metastasis rate of 10-20%). Histologically, it can be distinguished from atypical fibroxanthoma by the observation of subcutaneous tissue invasion, perineural invasion, and foci of necrosis.

Background Small series of ultrasound findings in dermatofibrosarcoma protuberans (DFSP) have been published, but the usefulness of this technique as a preoperative planning tool for tumor resection has not been studied. Materials and methods We retrospectively reviewed patients with DFSP at our hospital that underwent ultrasound examination. Depth of invasion was evaluated by ultrasound and histopathology. Accuracy of ultrasound for assessing depth of tumor invasion was estimated. Results Thirty histopathologically confirmed DFSPs were studied. Classic finger-like projections were observed in 73.3% of cases. A posterior hyperechoic area extending deep into the subcutaneous tissue correlated with the honeycomb DFSP pattern and was observed in 53.3% of patients. Concordance between ultrasound and histopathologic depth measurements was excellent. Lateral tumor extension and Doppler activity were not evaluated in our series. Conclusion Ultrasound showed excellent prediction of depth of invasion. Further studies are required to define the usefulness of ultrasound for determining lateral tumor extension.

Kaposi sarcoma is a vascular sarcoma with 4 clinical variants: classic Kaposi sarcoma, which mainly affect the extremities of elderly patients and follows a chronic, generally indolent course; African Kaposi sarcoma; immunosuppression-associated Kaposi sarcoma; and AIDS-associated Kaposi sarcoma. Type8 human herpesvirus is the etiologic agent in all 4variants. Cutaneous angiosarcoma is a cutaneous neoplasm with a very poor prognosis. It carries a high probability of local relapse and has a 10% to 15% survival rate at 5years. There are 3 main variants of cutaneous angiosarcoma: idiopathic angiosarcoma of the face and scalp; Stewart-Treves syndrome; and postradiation angiosarcoma. The only potentially curative treatment is surgery with or without radiotherapy. However, its indistinct borders and multicentric nature mean that treatment is often palliative with chemotherapy, radiotherapy, or both.

Introduction and objectives Vismodegib is the first selective Hedgehog inhibitor approved for the treatment of locally advanced and metastatic basal cell carcinoma (BCC). In this article, we describe our experience with the use of this drug to treat advanced and/or multiple BCCs at a cancer center over 5 years. Material and methods We analyzed the following variables: patient age and sex; tumor location, size, type, and characteristics; time since onset; primary or recurrent status; duration of treatment; response to treatment (complete, partial, stabilization, or absence of response); adverse effects; and recurrences. Results We treated 22 patients, of whom 20 had locally advanced BCCs and 2 had metastatic BCCs with lymph node involvement. The treatment was administered over a mean of 11.8 months. Nine patients (41%) achieved complete response and 10 (45%) partial response. The disease was stabilized in 3 (14%). Two patients relapsed after a median of 21 months. The main adverse effects were dysgeusia, alopecia, and muscle cramps, all of which were mild. None of the patients developed squamous cell carcinoma in an area treated with vismodegib, although metatypical changes were observed after treatment. Conclusions With a response rate of 96%, vismodegib is a safe and effective treatment for locally advanced BCC. Adverse effects are generally mild but they need to be taken into account owing to their high frequency.

Resumen Introduccion y objetivos Vismodegib es el primer inhibidor selectivo de la via de la senalizacion Hedgehog aprobado para el tratamiento del carcinoma basocelular (CBC) localmente avanzado y metastasico. Describimos nuestra experiencia en un centro oncologico con el vismodegib en el tratamiento de pacientes con CBC avanzados y/o multiples durante un periodo de 5 anos. Material y metodos Analizamos variables como la edad y el sexo del paciente, la localizacion, el tamano, el tipo y las caracteristicas del tumor, el tiempo de evolucion, si son tumores primarios o recidivas, la duracion del tratamiento, la respuesta a este (completa, parcial, estabilizacion o ausencia de respuesta), los efectos secundarios observados y las recidivas. Resultados Un total de 22 pacientes fueron tratados, 20 con CBC localmente avanzados y 2 con CBC metastasicos con afectacion ganglionar. El tratamiento fue administrado durante 11,8 meses de media. El 41% (9) de los pacientes obtuvieron una respuesta completa al tratamiento, un 45% (10) respuesta parcial y en el 14% (3) de los pacientes el tratamiento consiguio estabilizar la enfermedad. Tras una mediana de 21 meses, 2 casos recidivaron. Los principales efectos secundarios observados fueron disgeusia, alopecia y calambres musculares, todos ellos de caracter leve. Ningun paciente desarrollo un carcinoma epidermoide sobre el area tratada con vismodegib, aunque si cambios metatipicos tras el tratamiento. Conclusiones El vismodegib es un farmaco seguro y eficaz para el tratamiento del CBC localmente avanzado, con un porcentaje de respuesta del 86%. Los efectos adversos deben tenerse en cuenta por su alta frecuencia, aunque estos suelen ser de caracter leve.

Resumen Introduccion y objetivos El imiquimod es un excelente tratamiento para las queratosis actinicas, sin embargo, su pauta prolongada de 4 semanas y la reaccion local que produce pueden limitar su utilizacion. Los objetivos del estudio son demostrar la eficacia del imiquimod al 5% para las queratosis actinicas aplicado en una pauta continuada de 12 dias y correlacionar el grado de reaccion local con la eficacia. Pacientes y metodos Se incluyeron pacientes con al menos 8 queratosis actinicas que se trataron con imiquimod en crema al 5% durante 12 dias seguidos. Se evaluo la reaccion local como leve, moderada o intensa. El estudio estadistico de la correlacion entre la reaccion local y la respuesta clinica se realizo con la prueba χ2 de Pearson y el test de correlacion rho de Spearman. Resultados Un total de 65 pacientes completaron el estudio. Se obtuvo un 52,3% de respuestas completas y un 75,4% de respuestas parciales. Encontramos una asociacion estadisticamente significativa entre el grado de reaccion local y la respuesta al tratamiento tanto en la prueba χ2 de Pearson como en el test de correlacion rho de Spearman. Conclusiones La pauta continuada de imiquimod al 5% aplicado durante 12 dias es eficaz para el tratamiento de las queratosis actinicas. El grado de reaccion local durante el tratamiento se correlaciona con la respuesta clinica.

Basal cell carcinoma (BCC) represents the most commonly diagnosed human cancer among persons of European ancestry with etiology mainly attributed to sun-exposure. In this study we investigated mutations in coding and flanking regions of PTCH1 and TP53 and noncoding alterations in the TERT and DPH3 promoters in 191 BCC tumors. In addition, we measured CpG methylation within the TERT hypermethylated oncological region (THOR) and transcription levels of the reverse transcriptase subunit. We observed mutations in PTCH1 in 58.6% and TP53 in 31.4% of the tumors. Noncoding mutations in TERT and DPH3 promoters were detected in 59.2% and 38.2% of the tumors, respectively. We observed a statistically significant co-occurrence of mutations at the four investigated loci. While PTCH1 mutations tended to associate with decreased patient age at diagnosis; TP53 mutations were associated with light skin color and increased number of nevi; TERT and DPH3 promoter with history of cutaneous neoplasms in BCC patients. Increased reverse transcriptase subunit expression was observed in tumors with TERT promoter mutations and not with THOR methylation. Our study signifies, in addition to the protein altering mutations in the PTCH1 and TP53 genes, the importance of noncoding mutations in BCC, particularly functional alterations in the TERT promoter.

Basal cell carcinoma (BCC) represents the most commonly diagnosed human cancer among persons of European ancestry with etiology mainly attributed to sun-exposure. In this study we investigated mutations in coding and flanking regions of the PTCH1 and TP53 genes and noncoding alterations in the TERT and DPH3 promoters in 191 BCC tumors. In addition, we measured CpG methylation within the TERT hypermethylated oncological region (THOR) and transcriptions levels of the reverse transcriptase subunit. We observed mutations in PTCH1 in 59% and TP53 in 31% of the tumors. Noncoding mutations in TERT and DPH3 promoters were detected in 59% and 38% of the tumors, respectively. We observed a statistically significant co-occurrence of mutations at the four investigated loci. While PTCH1 mutations tended to associate with decreased patient age at diagnosis; TP53 mutations were associated with light skin color and increased number of nevi; TERT and DPH3 promoter with history of cutaneous neoplasms in BCC patients. TERT promoter mutations but not THOR methylation associated with an increased expression of the reverse transcriptase subunit. Our study signifies, in addition to the protein altering mutations in the PTCH1 and TP53 genes, the importance of noncoding mutations in BCC, particularly functional alterations in the TERT promoter.

There are no large series describing cutaneous histologic changes during treatment with vismodegib in locally advanced basal cell carcinoma (BCC).To analyze histologic changes in skin biopsy specimens from patients with locally advanced BCC treated with vismodegib.A descriptive, retrospective study of patients with locally advanced BCC treated with vismodegib between June 2012 and December 2017 at the Instituto Valenciano de Oncología, Spain. Nineteen patients were biopsied before and during the treatment with vismodegib, and we compared histologic changes observed.Seven patients (37%) achieved complete response, which was characterized by replacement of tumor stroma with a hyaline scar, lymphocytic inflammatory infiltrate, keratin formation, and infundibular cysts. Twelve patients (63%) achieved partial response; 5 showed no phenotypic changes, whereas 7 showed histologic changes; 5 cases showed metatypical differentiation; and 2 cases presented squamous differentiation. We observed no cases of squamous cell carcinoma arising at vismodegib treatment sites and no association between initial histologic subtype and clinical response.Many biopsy specimens were obtained by punch biopsy and may not be representative of the full tumors. We studied histologic changes only in complete and partial responses.Vismodegib can induce histologic changes toward metatypical or squamous differentiation of BCC in patients with partial response. Keratinizing phenomena were frequent, both in partial and complete response groups.

Resumen Introduccion y objetivo Actualmente existen cuestionarios para valorar el impacto en la calidad de vida en pacientes con cancer cutaneo. Sin embargo, no se cuenta con un instrumento especifico aplicable a casos con queratosis actinica. El objetivo de este estudio ha sido la traduccion y adaptacion transcultural del cuestionario Actinic Keratosis Quality of Life (AKQoL) al espanol. Pacientes y metodos El cuestionario original se tradujo al espanol de acuerdo con el modelo metodologico para la adaptacion transcultural de medidas autoadministradas. Se han calculado diversos indices para la fiabilidad general y la validez, incluyendo el coeficiente alfa de Cronbach y test-retest (correlacion de rango de Spearman y prueba de Bland-Altman), mientras que para la validez concurrente se utilizo el coeficiente de correlacion de Pearson entre los resultados de los cuestionarios AKQoL y Skindex-29. Resultados La version final se administro a un total de 621 sujetos con queratosis actinica con una puntuacion media ± desviacion estandar de 5,25 ± 4,73 (0 a 25 puntos). El analisis de fiabilidad mostro una alfa de Cronbach de 0,84. La correlacion entre el Skindex-29 (1,87 ± 4,07) y AKQoL (1,97 ± 2,98) fue de 0,344 (p = 0,002; rho de Spearman), con una proporcion de varianza compartida del 11,8%. Conclusiones Mediante los distintos procesos de traduccion, adaptacion transcultural y validacion en distintos grupos hemos generado una herramienta comprensible y fiable para valorar el impacto de la queratosis actinica en la calidad de vida de pacientes de nuestro entorno.

Background Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing cutaneous tumor that can invade the subcutaneous tissue, muscle tissue, and even bone. Objective To identify histologic features associated with greater depth of invasion, i.e., local aggressiveness, in DFSP. Material and methods We analyzed associations between histologic features of DFSP (e.g., type of subcutaneous invasion, histologic pattern, cell type, areas of fibrosarcoma) and the presence and absence of muscle fascia involvement. Results We studied 155 cases of DFSP. The following histologic characteristics were significantly associated with involvement of the muscle fascia: the presence of a sheetlike pattern, a high degree of cellular pleomorphism, and more than 1 mitotic figure. The tumor did not extend beyond the subcutaneous tissue in the majority of cases (62.6%), but there was involvement of the fascia or galea aponeurotica in 17 cases (11%) and of the muscle tissue in 36 cases (23.2%). Conclusions Histologic patterns, degree of pleomorphism, and number of mitotic figures are important predictors of deep invasion (fascia or muscle) in DFSP; these layers can be involved in up to 30% of cases.

Background: Treatment of oral erosive lichen planus is considered a therapeutic challenge. Various systemic and topical agents aimed at controlling the symptoms, rather than curing the lesions, have been used with varying results. Objective: To evaluate the response to treatment with antimalarial drugs in patients with oral erosive lichen planus. Methods: Eight patients diagnosed with oral erosive lichen planus were treated with antimalarial agents. The first clinical evaluation was made after a month of treatment and then every 2-3 months. Baseline ophthalmologic examinations were performed, and laboratory values were monitored before and during treatment. Results: All studied patients who had previously been resistant to other treatments responded favorably. Pain relief and reduced erythema and erosions were observed after of a mean of 2.4 months. Conclusion: Antimalarials may be useful for the treatment of oral erosive lichen planus. They are easily administered and affordable, with few adverse effects.

Introduction Daylight photodynamic therapy (PDT) is a new type of PDT that is as effective as conventional PDT in mild and moderate actinic keratosis but with fewer adverse effects, resulting in greater efficiency. The climatic conditions in the Iberian Peninsula require an appropriately adapted consensus protocol. Objective We describe a protocol for the treatment of grade I and II actinic keratosis with daylight-mediated PDT and methyl aminolevulinate (MAL) adapted to the epidemiological and clinical characteristics of Spanish and Portuguese patients and the climatic conditions of both countries. Methods Twelve dermatologists from different parts of Spain and Portugal with experience in the treatment of actinic keratosis with PDT convened to draft a consensus statement for daylight-mediated PDT with MAL in these countries. Based on a literature review and their own clinical experience, the group developed a recommended protocol. Results According to the recommendations adopted, patients with multiple mild and moderate lesions, particularly those at risk of developing cancer, are candidates for this type of therapy. Daylight PDT can be administered throughout the year, although it is not indicated at temperatures below 10 °C or at excessively high temperatures. Likewise, therapy should not be administered when it is raining, snowing, or foggy. The procedure is simple, requiring application of a sunscreen with a protection factor of at least 30 based exclusively on organic filters, appropriate preparation of the lesions, application of MAL without occlusion, and activation in daylight for 2 hours. Conclusion This consensus statement represents a practical and detailed guideline to achieve maximum effectiveness of daylight PDT with MAL in Spain and Portugal with minimal adverse effects.

Summary Background Dermatofibrosarcoma protuberans (DFSP) is an uncommon skin tumour with aggressive local growth. Whether DFSP should be treated with conventional surgery (CS) or Mohs micrographic surgery (MMS) has long been a topic of debate. Objectives To calculate, in a large series of DFSP treated by MMS, the minimum margin that would have been needed to achieve complete clearance by CS. Secondly, to calculate the percentage of healthy tissue that was preserved by MMS rather than CS with 2- and 3-cm margins. Methods The minimum margin was calculated by measuring the largest distance from the visible edge of the tumour to the edge of the definitive surgical defect. Tumour and surgical defect areas for hypothetical CS with 2- and 3-cm margins were calculated using AutoCAD for Windows. Results A mean minimum margin of 1·34 cm was required to achieve complete clearance for the 74 tumours analysed. The mean percentages of skin spared using MMS rather than CS with 2- and 3-cm margins were 49·4% and 67·9%, respectively. Conclusions MMS can achieve tumour clearance with smaller margins and greater preservation of healthy tissue than CS.

Introduction and objectives Imiquimod is an excellent option for patients with actinic keratosis, although its use may be limited by the long course of treatment required (4 weeks) and the likelihood of local skin reactions. The objectives of the present study were to demonstrate the effectiveness of a 12-day course of imiquimod 5% for the treatment of actinic keratosis and to examine the association between treatment effectiveness and severity of local reactions. Patients and methods We included patients with at least 8 actinic keratoses treated with imiquimod 5% cream for 12 consecutive days. Local reactions were classified as mild, moderate, or severe. The statistical analysis of the association between local reactions and clinical response was based on the Pearson χ2 test and the Spearman rank correlation test. Results Sixty-five patients completed the study. Complete response was recorded in 52.3% and partial response in 75.4%. We found a statistically significant association between severity of the local reaction and response to treatment in both the Pearson χ2 test and the Spearman rank correlation test. Conclusions A 12-day course of imiquimod 5% proved effective for the treatment of actinic keratosis. Severity of local reactions during treatment was correlated with clinical response.

Dermatofibrosarcoma protuberans (DFSP) is a rare superficial tumor characterized by high rates of local recurrence and low risk of metastasis. DFSP occurs most commonly on the trunk and proximal extremities, affects all races, and often develops between the second and fifth decade of life. The tumor grows slowly, typically over years. Histologically, several variants of DFSP have been described and should be well characterized to avoid misdiagnosis with other tumors. These include pigmented (Bednar tumor), myxoid, myoid, granular cell, sclerotic, atrophic DFSP, giant cell fibroblastoma, and DFSP with fibrosarcomatous areas. Of all these variants, only the DFSP with fibrosarcomatous areas is high grade, with a higher rate of local recurrence and distant metastasis. DFSP is genetically characterized by the t(17;22)(q22;q13), resulting in the fusion of alpha chain type 1 of collagen gene and platelet-derived growth factor beta gene. This translocation is present in 90% of DFSP and represents a very useful tool in the differential diagnosis of DFSP with other tumors with similar histology. The standard treatment is wide local excision with at least a 2-cm margin. However, local recurrence after apparently adequate surgical excision is well recognized. Mohs micrographic surgery would be the treatment of choice with a better cure rate and maximal conservation of tissue. When surgery is insufficient, clinical evidence has suggested that imatinib mesylate is a safe and effective treatment in DFSP, especially in cases of local advanced or metastatic disease. This article presents an overview of the state of the art in the clinicopathological management of this disease.

Resumen Introduccion Los carcinomas cutaneos agresivos que aparecen en el cuero cabelludo se desarrollan habitualmente en pacientes de edad avanzada que presentan multiples comorbilidades. La exeresis completa de estos tumores incluye en muchos casos el periostio, lo que conlleva que la reconstruccion de este tipo de defectos se convierta en un reto. Objetivo Se evalua la utilidad de un parche de colageno porcino tipo I como tratamiento coadyuvante o definitivo en el cierre quirurgico de este tipo de defectos cutaneos. Material y metodos Se realizo un estudio prospectivo en el que se incluyeron aquellos pacientes que presentaron defectos de mas de 5 cm en el cuero cabelludo, en los que el periostio tuvo que ser extirpado para alcanzar margenes libres de enfermedad entre enero de 2009 y noviembre de 2011. Resultados La tumoracion mas prevalente fue el carcinoma epidermoide cutaneo recurrente. El defecto posquirurgico oscilo entre 5 y 7 cm de diametro. En el grupo de casos en los que se coloco un injerto posparche (n = 4), en el 100% de los casos el tejido prendio completamente. En el caso de aquellos pacientes en los que el parche fue el recurso definitivo (n = 6) el tejido de granulacion generado por el material biosintetico de colageno porcino ocupo el defecto quirurgico, dejandolo completamente restituido en aproximadamente 3,5 meses. Conclusiones El parche de colageno porcino tipo i es una herramienta sencilla, barata y eficaz en el tratamiento coadyuvante o definitivo del cierre de defectos quirurgicos del cuero cabelludo de mas de 5 cm que carecen de periostio.

Introduction: Surgical excision with margins of 0.5 cm is the standard treatment for lentigo maligna (LM). Excision, however, is often incomplete as many of these tumors have indistinct borders. Objective: To identify clinical predictors of subclinical extension in primary and recurrent LM of the head and thereby determine which lesions might require wider surgical margins. Material and methods: We reviewed the clinical records of patients with LM of the head treated definitively with conventional surgical excision or slow micrographic Mohs surgery (MMS) at the dermatology department of Instituto Valenciano de Oncologia between January 1993 and April 2011. Results: Surgical margins larger than 0.5 cm were required in 69.2% of recurrent LM and 26.5% of primary LM. Factors associated with the need for wider margins were prior treatment that might have interfered with the clinical delineation of the border, lesions in the center of the face, and skin phototypes III to V. Conclusions: Surgical margins of 0.5 cm are inadequate for the treatment of a considerable number of LM lesions located on the head, particularly if these are recurrent. Slow MMS using paraffin-embedded sections appears to be the treatment of choice in such cases, particularly for recurrent lesions or lesions with poorly defined borders or possible subclinical extension.

Resumen Introduccion El tratamiento estandar del lentigo maligno (LM) es la escision quirurgica con margenes de 0,5 cm. Sin embargo, dada la mala delimitacion de muchos tumores, es frecuente que esta exeresis sea incompleta. Objetivo identificar parametros clinicos que puedan predecir que LM localizados en la cabeza, extirpados de forma primaria o tras recidivar, se extienden mas alla de los limites visibles y por tanto, puedan requerir margenes quirurgicos mas amplios. Material y metodos se reviso retrospectivamente la informacion clinica de los pacientes con LM localizado en la cabeza cuyo tratamiento quirurgico definitivo, mediante cirugia convencional o cirugia de Mohs diferida, fue realizado en el Servicio de Dermatologia del Instituto Valenciano de Oncologia (IVO) entre enero de 1993 y abril de 2011. Resultados un 69,2% de los LM recidivados y un 26,5% de los tumores primarios requirieron margenes de mas de 0,5 cm. La administracion previa de tratamientos que puedan interferir en la delimitacion clinica, la localizacion centrofacial y las lesiones que se presentan en pacientes con fototipos altos (III-V) fueron los factores asociados a la necesidad de margenes quirurgicos mas amplios. Conclusiones la utilizacion de margenes de 0,5 cm para el tratamiento del LM es insuficiente para un numero importante de casos localizados en la cabeza, especialmente los recidivados. La cirugia de Mohs diferida, con el estudio de todos los margenes en parafina, parece el tratamiento de eleccion en particular para los casos recidivados o en los que la delimitacion clinica pueda verse dificultada.

The different features of spitzoid melanoma are not well characterized in the literature, and the lesion often has to be described in comparison with Spitz nevus. We evaluated the histopathological appearance of spitzoid melanoma by reviewing all spitzoid melanomas treated at our hospital and all referrals from 1998 to 2010. The final study sample comprised 18 cases, 11 from our institution and 7 referrals from other centers. We recorded clinical parameters (eg, age, sex, site, time between onset and excision, recurrence, and death) and a series of histopathological parameters (eg, size, ulceration, symmetry, Clark level, Breslow thickness, cell density, atypia, mitosis). Clinical and histopathological criteria were not available for the 7 referrals. Mean age was 35.2 years (15-56), and 8 patients were women. Mean size of the lesions was 7.27 mm (Clark III/IV and Breslow 2.51 mm), and these were found on the limbs and trunk. Cell density was high in 10 cases and atypia present in 9 (marked in 1). Mitoses were observed in 8 cases (atypical in 4, clusters in 4). Maturation was absent in 9 cases and zonation in 8. Our analysis revealed 5 previously undefined subtypes of spitzoid melanoma (genuine (7 cases), uniform (5 cases), packed (5 cases), polypoid (3 cases) and pigmented (2 cases)]. Four cases showed 2 patterns at the same time. The most useful parameters for the differential diagnosis were cell density, mitosis, zonation, infiltration pattern, and consumption of the epidermis. Assignation of a spitzoid melanoma to 1 of more of our 5 subtypes can enable a more confident diagnosis to be made.

Resumen La terapia fotodinamica (TFD) y el imiquimod son dos excelentes tratamientos utilizados frecuentemente en Dermatologia para las queratosis actinicas, el carcinoma basocelular (CBC) o la enfermedad de Bowen. No existen suficientes estudios comparativos entre ellos para poder extraer buenas conclusiones sobre su eficacia, su tolerancia o sus efectos secundarios y para poder situar a un tratamiento por encima del otro en un tipo de lesion o paciente en concreto. Por otra parte, existen situaciones o indicaciones particulares como la queilitis actinica, los pacientes inmunodeprimidos o los CBC localizados en parpados donde estos dos tratamientos pueden considerarse inicialmente contraindicados; sin embargo, existe suficiente evidencia para poder utilizarlos. Vamos a realizar una revision de la TFD y el imiquimod, bajo un punto de vista critico y comparativo entre ellos, para poder ayudar a responder a la cuestion de que tratamiento es mas recomendable en un paciente determinado.

Background Photodynamic therapy (PDT) and imiquimod are the treatments of choice for actinic keratosis (AK). As they have different mechanisms of action, it seems reasonable to assume that applying both treatments sequentially would be efficacious. Objectives We sought to determine which of these therapeutic modalities provides a better clinical and histologic response in patients with AK and whether sequential use of both was more efficacious than each separately. Methods Patients were randomly assigned to one treatment group: group 1, PDT only; group 2, imiquimod only; or group 3, sequential use of PDT and imiquimod. The primary outcome measure was complete clinical response. Partial clinical response was defined as a reduction of more than 75% in the initial number of lesions. A complete clinicopathologic response was defined as lack of evidence of AK in the biopsy specimen. Results In all, 105 patients completed the study (group 1, 40 patients; group 2, 33 patients; group 3, 32 patients). Sequential application of PDT and imiquimod was more efficacious in all the outcome measures. More patients were satisfied with PDT than with the other two modalities ( P = .003). No significant differences were observed among the 3 modalities and tolerance to treatment. Limitations Only one cycle of imiquimod was administered. The follow-up period was brief. Conclusions Sequential application of PDT and imiquimod provides a significantly better clinical and histologic response in the treatment of AK than PDT or imiquimod monotherapy. It also produces less intense local reactions and better tolerance and satisfaction than imiquimod monotherapy.

Background Management of facial extensive recurrent basal cell carcinoma can be a challenge for dermatologists. Although the preferred technique is usually Mohs surgery, sometimes the patient’s condition or predicted aggressive surgery make other options advisable. Methods We describe a case of a giant recurrent basal cell carcinoma in the face of an old woman successfully treated by combined therapy with MAL-photodynamic therapy and topical 5%. Results The patient remains well and with no sign of the tumor, with very good cosmetic result two years after treatment. Conclusions Management of extensive facial basal cell carcinoma with combined therapies, as photodynamic therapy followed by topical imiquimod, can be an option for selected cases such as ours.

Resumen Introduccion La actitud ante los carcinomas basocelulares que han sido extirpados con margenes afectos es controvertida en la literatura. Frente a casos que no recidivan incluso tras muchos anos de seguimiento, hay riesgo de complicaciones en aquellos que si lo hacen. Podrian existir factores que ayuden a escoger la mejor opcion terapeutica para los carcinomas basocelulares localizados en la cabeza y extirpados con margenes afectos. Material y metodos Se seleccionaron 46 pacientes tratados mediante cirugia de Mohs por haber sido previamente extirpados con afectacion de margenes. Se valoro que factores se asociaron a la ausencia de restos tumorales y que factores estaban asociados a la obtencion de margenes negativos con un unico estadio. Resultados No se consiguio ninguna relacion significativa con el sexo, el tamano tumoral, el margen que estaba afecto (lateral, profundo o ambos), el tiempo de evolucion, el numero de tratamientos previos, el tipo histologico ni la localizacion. Hubo una cierta tendencia a encontrar mas frecuentemente restos tumorales en las areas perioficiales de tumores con ambos margenes afectos, y a necesitar un unico estadio en los tumores de menos de 1,2 cm y con menos de 5 anos de evolucion. Conclusiones No existen datos concluyentes que permitan definir en que casos puede no ser necesaria la reextirpacion ni en cuales puede ser suficiente una exeresis convencional. La mejor opcion para el tratamiento de estos tumores es la cirugia de Mohs, aunque podria ser razonable realizar exeresis convencional en tumores pequenos de poco tiempo de evolucion en localizaciones de bajo riesgo.

Background There is debate in the literature regarding the management of basal cell carcinoma following excision with positive surgical margins. While in some cases recurrence is not observed even after many years of follow-up, those in which recurrence does occur are at an increased risk of complications. Factors may exist that help to choose the best therapeutic approach for basal cell carcinomas on the head in which positive margins are observed following excision. Material and methods A total of 46 patients were selected who had been treated by Mohs micrographic surgery as a result of positive surgical margins being present following previous tumor excision. The factors associated with the absence of tumor nests and the occurrence of negative margins following a single Mohs stage were analyzed. Results No associations were observed with sex, tumor size, affected margin (lateral, deep, or both), time since diagnosis, number of previous treatments, histological type, or tumor site. There was a certain trend towards more frequent identification of tumor remnants when both surgical margins were affected and towards a requirement for a single Mohs stage in tumors less than 1.2 cm and in which less than 5 years had elapsed since diagnosis. Conclusions Conclusive data are unavailable with which to define cases in which re-excision is necessary or those in which conventional excision could be sufficient. The best option for the treatment of these tumors is Mohs micrographic surgery, although conventional excision could be reasonable in small tumors located at low-risk sites and in which long periods have not elapsed since diagnosis.

Dermatofibrosarcoma protuberans (DFSP) is characterized by asymmetrical and poorly defined growth. Magnetic resonance imaging (MRI) has been proposed for the delimitation of this tumor.To study the utility of MRI in evaluating the depth of infiltration in DFSP and to compare the efficiency of clinical palpation with that of MRI in delimiting the invasiveness of DFSP.Observational, prospective study of DFSP cases. The MRI scans for all cases were compared with the exact histological infiltration plane obtained using modified Mohs micrographic surgery (MMS).Forty-three DFSPs were included: 22 primary, nine recurrent, and 12 extirpated with positive margins. Sensitivity for detecting deep invasion was 58% on examination using palpation and 67% using MRI.We present the largest series of DFSP cases studied using MRI published to date. In primary cases, MRI has greater sensitivity than palpation for detecting depth of infiltration (67% vs 58%). MRI seems to be useful in primary DFSP in locations other than the head, neck, and upper part of the thorax. MRI is not useful for confirming tumor persistence in extirpated DFSP with positive margins or for studying lateral extension in primary DFSP.

Resumen Introduccion El queratoacantoma es considerado hoy dia un carcinoma epidermoide in situ que aparece principalmente en pacientes mayores de 70 anos. Se trata de un tumor de buen pronostico que, en algunos casos, muestra resolucion espontanea. El tratamiento de este tipo de tumoracion es la exeresis simple. Sin embargo, la localizacion preferente en las regiones facial y acral, el tamano y su rapido crecimiento son factores que hacen que la cirugia sea en algunos casos agresiva y antiestetica. Objetivo El objetivo principal del estudio es evaluar la eficacia de la infiltracion intralesional de metotrexato en la reduccion del tamano prequirurgico de la lesion y del correspondiente defecto quirurgico resultante de la intervencion. Material y metodos Se realizo un estudio prospectivo aleatorizado en el que se incluyeron todos aquellos pacientes atendidos en nuestro Servicio diagnosticados de queratoacantoma de al menos 1,5 cm de tamano entre enero de 2009 y enero de 2010. Se establecieron dos grupos, uno en el que los pacientes recibieron una infiltracion de metotrexato previamente al acto quirurgico y otro en el que se realizo directamente la cirugia. Resultados De los 25 pacientes incluidos en el estudio, 10 casos recibieron neoadyuvancia con metotrexato intralesional (grupo A) y 15 casos fueron intervenidos mediante cirugia aislada (grupo B). Los pacientes del grupo A mostraron una reduccion en el tamano tumoral en el momento de la cirugia que oscilo entre un 50 y un 80%. Ninguno de los pacientes presento complicaciones relacionadas con la inoculacion del metotrexato ni con la intervencion quirurgica. En el grupo B solo uno de los casos mostro una discreta disminucion de sus dimensiones en el momento del acto quirurgico. El resto de las lesiones mostraron una estabilidad (4 casos; 26%) e incluso un aumento de las dimensiones del tumor (10 casos; 66%) en el momento de la intervencion. Cinco de los casos incluidos en este ultimo grupo requirieron ingreso hospitalario en relacion con la intervencion quirurgica. Conclusiones El metotrexato intralesional como terapia neoadyuvante es una medida bien tolerada, que permite evitar cirugias agresivas en pacientes de edades avanzadas que presentan un queratoacantoma de diametro superior a 1,5 cm localizado en la region facial y acral.

Background Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor, usually low grade, except for the fibrosarcomatous variant (DFSP-FS). Objectives We sought to compare the clinicopathological, immunohistochemical, genetic, and therapeutic features between DFSP and DFSP-FS. Methods The clinicopathological features were reviewed in 63 DFSP and 12 DFSP-FS. Immunohistochemistry and multiplex reverse transcriptase-polymerase chain reaction were carried out using formalin-fixed, paraffin-embedded tissue, using specific primers for collagen type I alpha 1 ( COL1A1 ) and platelet-derived growth factor beta ( PDGFB ). Results DFSP-FS was associated with tumor history longer than 5 years ( P = .009), tumor size greater than 4 cm ( P = .001), more stages of modified Mohs micrographic surgery ( P = .005), expansive subcutaneous infiltration ( P = .005), muscular invasion ( P = .0001), absence of CD34 staining ( P = .018), p53 positivity ( P = .006), and increased proliferative activity ( P = .004) compared with DFSP. The COL1A1-PDGFB fusion transcript was found in 100% DFSP-FS and 72% DFSP. No association was found between the different COL1A1-PDGFB fusion transcripts and the different histologic subtypes. Wide local excision (2 cm) was performed in 47% of cases and modified Mohs micrographic surgery in 53%. After a mean follow-up of 73 months (range 21-235), 6 patients had local recurrence (5 DFSP, 1 DFSP-FS) and one died of disease (DFSP-FS). The only factor related to local recurrence was the type of surgery (17% wide local excision vs 0% modified Mohs micrographic surgery) ( P = .006). Limitations Our study is retrospective. Prospective studies are necessary to confirm our results. Conclusions DFSP-FS reflects tumor progression in DFSP, with larger size, particular invasive patterns, p53 expression, and increased proliferative activity. However, as in low-grade DFSP, appropriate surgery permits a tumor-free excision. COL1A1-PDGFB is a useful tool for diagnosis of DFSP and particularly for DFSP-FS.

The growth rate (GR) of melanomas is not uniform. A fast-growing subtype has been identified and seems to have a role in the stabilization of the mortality rates because of melanoma. To examine features associated with fast-growing melanomas (FGMs) and to determine the relationship between the GR and well-recognized prognostic factors of melanoma, a series of 386 new invasive cutaneous melanomas seen during 2004-2009 were retrieved from our database. The GR was calculated according to earlier published studies. FGMs were defined as those whose GR was greater than 0.49 mm per month. Differences in clinical, epidemiological, and pathological features were evaluated. Correlations between the GR, tumor thickness, and mitotic rate were also analyzed. FGMs were significantly more prevalent among patients aged over 65 years and with a higher rate of past personal history of nonmelanoma skin cancer. This subtype was over-represented among melanomas located on both nonexposed and usually exposed skin and was less related to earlier sunburns. Patients with FGMs presented with more aggressive pathological features and had more advanced disease with sentinel node analysis affected in up to 35% of cases. There was a strong positive correlation between the GR and tumor thickness (r=0.762), and mitotic rate (r=0.542). This study was limited by being retrospective in nature. FGMs are a highly aggressive subtype of melanomas that seem to develop after at least two routes, one related to chronic sun exposure and another unrelated to the sun. Older patients have a higher predisposition to develop this kind of tumor. This variant warrants specific strategies to improve primary and secondary prevention.

Summary Background Photodynamic therapy (PDT) and imiquimod are two excellent treatments for actinic keratosis but are often not well tolerated by patients. Objectives To ascertain which treatment is better tolerated and which produces greater patient satisfaction. A secondary objective was to determine the factors related to the patient’s tolerance to each treatment. Methods Patients with at least five actinic keratosis lesions on the face and scalp were selected. The patients were randomized to receive treatment with PDT with methyl aminolaevulinate or treatment with imiquimod. Tolerance, satisfaction and predisposition to repeat the treatment were evaluated. Results Most patients exhibited good or acceptable tolerance to both PDT and imiquimod treatment. There was a higher percentage of patients treated with PDT (93%) who were very satisfied compared with imiquimod (62%) (P = 0·004). Most patients treated with either one of the two options would repeat the same treatment. No significant relationship was found between age, sex, working time exposed to the sun, phototype and hair colour and the tolerance to both treatments. Conclusions Both PDT and imiquimod are treatments that are generally well tolerated. While both treatments provide a high level of satisfaction, PDT appears to be slightly superior in this regard.

Background Human papilloma virus (HPV) is increasingly recognized as an important human carcinogen but its role in the aetiopathogenesis of basal cell carcinoma (BCC) in immunocompetent individuals is unclear. Objective A prospective case–control study was designed to compare the prevalence of HPV in BCC and normal skin samples from immunocompetent subjects and to assess the influence of different clinical features on the risk of cutaneous HPV. Methods A total of 142 samples from 70 BCC cases (superficial BCC 38 and nodular BCC 32) and 72 controls were analysed by a degenerated nested PCR technique. Clinical data were recorded and risk factors for HPV infection were assessed by multivariate analysis. Results There were 31 HPV DNA-positive samples. HPV was detected more frequently in cases (25.7%) than in controls (18.1%) and in nodular (31.3%) than in superficial (21.1%) BCC lesions but differences were not statistically significant. Older age (OR 1.5; 95% CI: 1.02–1.09) and actinic keratosis (OR 2.62; 95% CI 1.15–5.96) were the only significant factors associated to the presence of HPV. Risk of HPV positivity was also higher in blond-haired subjects, fair/pale skin colour, history of sunburn, solar lentigines and seborrheic keratosis but the differences were not significant. Both in cases and controls, β -types were the most frequent. Conclusions HPV does not seem to play a fundamental role in the aetiopathogenesis of either nodular or superficial BCC. The presence of HPV appears to be more related to actinic damage and possibly to an alteration of the barrier function associated with ageing.

Resumen Antecedentes Los pacientes diagnosticados de un melanoma cutaneo tienen mas riesgo que el resto de la poblacion de padecer nuevos melanomas a lo largo de su vida. La mayoria de estos melanomas aparecen «de novo», pero existe un porcentaje de ellos que se desarrolla sobre nevos melanociticos previos. Objetivo Determinar los factores de riesgo asociados al desarrollo de un segundo melanoma cutaneo sobre nevo en pacientes diagnosticados de melanoma cutaneo. Metodos Se seleccionaron los datos de 981 pacientes diagnosticados de un primer melanoma cutaneo, 47 de los cuales habian sido diagnosticados de un segundo melanoma. Todos los pacientes fueron clasificados en funcion de si el melanoma presento o no asociacion histologica a un nevo. Resultados La edad inferior a 40 anos, la localizacion en el tronco y el subtipo histologico de extension superficial, fueron los factores de riesgo independientes asociados al desarrollo de un primer melanoma sobre nevo. El unico factor asociado al desarrollo de un segundo melanoma sobre nevo fue que el primer melanoma tambien se hubiera presentado asociado a un nevo (OR: 9,51, 95% de intervalo de confianza: 1,6–56,56; p=0,042). Conclusiones Los melanomas se desarrollan asociados a un nevo principalmente en pacientes jovenes, en el tronco y del subtipo tipo de extension superficial. A su vez, el desarrollo de un primer melanoma sobre nevo aumenta el riesgo hasta 9 veces de desarrollar un segundo melanoma sobre un nevo previo. Estos hallazgos, subrayan la importancia del seguimiento estrecho de todas las lesiones melanicas en los pacientes que han desarrollado un primer melanoma sobre nevo.

Introduction and objectives Microcystic adnexal carcinoma is a rare and aggressive tumor that manifests clinically as a subcutaneous nodule located on the head or neck. The tumor can be confused clinically and histologically with other benign and malignant skin lesions, often leading to inappropriate initial treatment. The chief concern with microcystic adnexal carcinoma is the elevated morbidity and the high rate of recurrence after wide local excision. Recent preliminary studies point to higher cure rates with Mohs micrographic surgery. Material and methods We reviewed the medical histories of 6 consecutive patients with microcystic adnexal carcinoma who underwent Mohs micrographic surgery in our dermatology department between 1995 and 2007. Results In all cases, lesions were located on the head and were primary tumors. Seventy percent of the tumors were wrongly diagnosed initially as basal cell carcinoma. Perineural invasion was not detected in any patient, and all were free of recurrence after between 1 and 12 years of postoperative follow-up. Conclusions The absence of perineural involvement and substantial cell atypia can be attributed to the lesions being primary tumors. This would provide a rationale for definitive radical treatment of the primary tumor from the outset to avoid the complications associated with recurrence. The site and the absence of recurrence in all our patients who underwent Mohs micrographic surgery support the use of this technique as the treatment of choice in microcystic adnexal carcinoma.

Summary Background Photodynamic therapy (PDT) is an effective treatment for actinic keratoses, Bowen’s disease and basal cell carcinoma. The main drawback of PDT is pain during application. Objectives To compare the efficacy of supratrochlear and supraorbital nerve block with cold air analgesia to control the pain experienced during PDT. Methods A controlled open clinical trial was conducted in 34 patients having multiple actinic keratoses in the frontal region treated with PDT. On one side of the frontal region the supratrochlear and supraorbital nerves were blocked, while on the other side cold air was used as the method of analgesia. Pain was recorded on a visual analogue scale after treatment. Results Thirty-one of 34 patients reported less pain in the zone treated with nerve block. This difference was statistically significant. Conclusions Nerve block is superior to cold air and is an easy, safe, effective means of controlling the pain associated with PDT.

Resumen Introduccion Se ha observado que los melanomas tienen un perfil genetico similar segun el patron de exposicion al sol del area cutanea donde asientan. El objetivo de este estudio ha sido caracterizar desde el punto de vista clinico los pacientes con melanoma segun su patron de exposicion al sol: cronico, intermitente y ausencia de exposicion. Material y metodos Se seleccionaron 789 pacientes con melanoma diagnosticados en nuestro centro de forma consecutiva desde enero del ano 2000 a partir de la base de datos de melanoma. Se obtuvieron los datos referentes a las caracteristicas epidemiologicas, fenotipicas y los antecedentes personales y familiares de cancer. Se compararon las frecuencias observadas de cada una de las variables estudiadas. Resultados Se observo que la mayoria de los pacientes tienen los melanomas en localizaciones expuestas al sol de forma intermitente. Ademas, estos pacientes tienen mas nevus melanociticos tanto comunes como atipicos y su melanoma se asocia con una elevada frecuencia a un nevus preexistente. Por otra parte, el segundo grupo en numero esta constituido por el grupo de pacientes con melanomas en areas cutaneas expuestas al sol de forma cronica que presentaron todas las lesiones (lentigos solares y queratosis actinicas) y antecedentes tipicos de esta localizacion. Finalmente, el grupo de melanomas que se presenta en areas de piel no expuestas al sol tiene, al igual que el anterior, una edad de diagnostico mayor y se diagnostican en fases avanzadas de la enfermedad. Conclusiones La presencia de un elevado numero de pacientes que no sigue ninguno de los patrones mencionados suscita la necesidad de caracterizarlos clinica y biologicamente para conocer vias alternativas de desarrollo de un melanoma, y asi poder establecer medidas preventivas adecuadas.

Resumen Introduccion El eritema acral (EA) es una reaccion cutanea relativamente frecuente producida por diferentes agentes quimioterapicos. Otros terminos con los que se le conoce son eritrodisestesia palmoplantar o sindrome pie-mano. Se presenta como un eritema doloroso en palmas y plantas asociado a parestesias en el contexto de un tratamiento oncologico. El EA parece ser dosis-dependiente, y tanto el pico plasmatico como la dosis acumulada del quimioterapico determinan su aparicion. La clinica y los hallazgos histopatologicos sugieren una citotoxicidad directa de la epidermis acral por las altas concentraciones de los quimioterapicos. Los agentes mas frecuentemente implicados son doxorrubicina, 5-fluorouracilo y derivados, citarabina y docetaxel. Material y metodos Se presentan las caracteristicas clinicas e histologicas de una serie de pacientes diagnosticados de eritema acral por quimioterapicos. Se incluyeron en el trabajo todos los pacientes sometidos a quimioterapia que desarrollaron lesiones de eritema acral durante un periodo de tiempo comprendido entre enero de 2000 y diciembre de 2003. Resultados y conclusion Se encontraron 44 casos entre los 2.186 pacientes sometidos a quimioterapia, lo que supuso una incidencia del 2,01 % durante el periodo de estudio, y el 16,75 % de todas las lesiones cutaneas atribuidas a la quimioterapia. El farmaco mas frecuentemente relacionado fue el 5-fluorouracilo en infusion continua, y la mayor incidencia se dio en pacientes tratados con doxorrubicina liposomial. El EA fue toxicidad limitante de dosis en el 29,5% de los casos. Los hallazgos histologicos variaron segun la intensidad clinica de las lesiones, encontrando una dermatitis de interfase con necrosis de queratinocitos variable, dilatacion del plexo vascular superficial y un escaso infiltrado inflamatorio. El tratamiento mas utilizado fue la piridoxina y las medidas locales como fomentos frios, emolientes y corticoids topicos.

Introduction. Acral erythema, also known as palmoplantar erythrodysesthesia or hand-foot syndrome, is a relatively common cutaneous reaction caused by a variety of chemotherapeutic agents. It presents during cancer treatment as painful erythema and paresthesia affecting the palms and soles. It seems to be dose dependent and its appearance is determined by both the peak plasma concentration and the cumulative dose of the chemotherapeutic agent. The symptoms and histopathology findings are suggestive of direct cytotoxicity affecting the epidermis of the extremities caused by high concentrations of chemotherapeutic agents. The most commonly implicated agents are doxorubicin, 5-fluoracil and its derivatives, cytarabine, and docetaxel. Material and methods. We present the clinical and histologic characteristics of a series of patients diagnosed with chemotherapy-induced acral erythema. The study included all patients who developed acral erythema lesions following chemotherapy between January 2000 and December 2003. Results and conclusions. Out of 2186 patients who underwent chemotherapy, 44 cases of acral erythema were identified, representing an incidence of 2.01% during the study period and 16.75% of all cutaneous lesions attributed to chemotherapy. The most commonly implicated drug was 5-fluoracil administered by continuous infusion and the highest incidence was observed in patients treated with liposomal doxorubicin. Acral erythema was a dose-limiting toxic effect in 29.5% of cases. The histologic findings varied according to the clinical severity of the lesions and included interface dermatitis with variable keratinocyte necrosis, dilation of the superficial vascular plexus, and limited inflammatory infiltrate. The most commonly used treatment was pyridoxine, along with topical treatments such as cold compresses, emollients, and topical corticosteroids.

Stewart-Bluefarb syndrome is a rare condition involving skin lesions that share clinical features with Kaposi sarcoma and that are secondary to an underlying arteriovenous fistula. We report the case of a 24-year-old man with progressive growth of skin lesions on the lower third of his left leg. Diagnosis of Stewart-Bluefarb syndrome was confirmed histologically and with detection of an arteriovenous fistula.

Dermatofibroma is a very frequent lesion that usually appears as a slowly growing nodule in the dermis, and preferentially involves the lower extremities of women. Several clinical variants have been described. Giant dermatofibroma has been defined as a rare variant of dermatofibroma measuring more than 5 cm that presents typical histological features and a benign biological behavior. We report the case of a 52-year-old man that presented a giant dermatofibroma with a diameter of 6 cm in the right shoulder and we review the few cases of this variant that have been described in the literature.