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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.

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 El dermatofibrosarcoma protuberans (DFSP) es un raro tumor cutaneo de crecimiento lento e infiltrativo que alcanza el tejido celular subcutaneo, el tejido muscular e incluso el hueso. Objetivos Buscar las caracteristicas histologicas asociadas a una mayor agresividad local en los DFSP, en forma de afectacion en profundidad. Material y metodos Se relaciono las caracteristicas histologicas propias del DFSP (forma de infiltrar el tejido celular subcutaneo, patron histologico, tipo celular, areas de fibrosarcoma) con la presencia o ausencia de afectacion de la fascia muscular. Resultados Se incluyeron 155 casos de DFSP. Las caracteristicas histologicas asociadas significativamente con la afectacion de la fascia muscular fueron: el patron histologico en sabana, un alto grado de pleomorfismo celular y la presencia de mas de una mitosis. En la mayoria de los casos (62,6%) el tumor se limito al tejido celular subcutaneo, en 17 casos (11%) contacto con la fascia muscular o con la galea aponeurotica, y en 36 casos (23,2%) afecto al tejido muscular. Conclusiones Es importante tener en cuenta el patron histologico, el pleomorfismo y el numero de mitosis en los DFSP para predecir su afectacion en profundidad (fascia o musculo), que puede llegar al 30% de los casos.

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.

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.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.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.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.

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 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, slow-growing cutaneous tumor that can invade the subcutaneous tissue, muscle tissue, and even bone.To identify histologic features associated with greater depth of invasion, i.e., local aggressiveness, in DFSP.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.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%).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.

Dermatofibrosarcoma protuberans (DFSP) typically affects the dermis and subcutaneous tissue. The subcutaneous variant is rare.We sought to characterize the subcutaneous DFSP (SC-DFSP) variant and compare it with cutaneous DFSP (C-DFSP).This work was a retrospective study of DFSP treated in our institution.Of 124 cases of DFSP, 18 were SC-DFSP (14.5%). Except for the deep location, the pathologic and genetic features were indistinguishable from the C-DFSP variant. Histologically, of 18 SC-DFSP cases, 13 were classic DFSP, 3 fibrosarcomatous DFSP (FS-DFSP), 1 Bednar tumor, and 1 giant-cell fibroblastoma. All tumors expressed CD34 and the COL1A1-PDGFB fusion transcripts. In our series, higher proportions of SC-DFSP tumors (61%) than C-DFSP tumors (8.5%) were located on the head (P .001). Of the 20 DFSP tumors on the head (16.1%), 11 were SC-DFSP and 9 were C-DFSP. In addition, half the SC-DFSP tumors affected muscle or periosteum, compared with a quarter of the C-DFSP tumors (P = .009). SC-DFSP needed a higher number of Mohs stages than did C-DFSP (P = .009). Median follow-up time was 63 months, and 2 FS-DFSP tumors recurred (1 SC-DFSP, 1 C-DFSP).Limitations include the retrospective aspect of the study.Most DFSP tumors involving the head were subcutaneous and required more complex surgery. Dermatologists should be aware of this atypical presentation, especially in lesions involving the head.