Experience With Bexarotene to Treat Cutaneous T-Cell Lymphomas: A Study of the Spanish Working Group of Cutaneous Lymphomas. / [Artículo traducido] Experiencia con bexaroteno en linfoma cutáneo de células T: un estudio del Grupo Español de Linfomas Cutáneos (GELC).

BACKGROUND AND OBJECTIVES: Bexarotene has been approved to treat advanced stage cutaneous T-cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL. MATERIAL AND METHOD: This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or Sézary syndrome (42) on a 10-year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals. RESULTS: A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27-95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1-114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment-related grade 5 adverse events were reported. CONCLUSIONS: Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL.

Experience With Bexarotene to Treat Cutaneous T-Cell Lymphomas: A Study of the Spanish Working Group of Cutaneous Lymphomas.

BACKGROUND AND OBJECTIVES: Bexarotene has been approved to treat advanced stage cutaneous T-cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL. MATERIAL AND METHOD: This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or Sézary syndrome (42) on a 10-year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals. RESULTS: A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27-95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1-114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment-related grade 5 adverse events were reported. CONCLUSIONS: Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL.

Hipoqueratosis plantar circunscrita / Circumscribed Plantar Hypokeratosis

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Lupus eritematoso con afectación genital: una localización inusual / Lupus Erythematosus Affecting the Genitalia: An Unusual Site

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Alopecia frontal fibrosante en el varón: presentación de 12 casos y revisión de la literatura / Frontal fibrosing alopecia in men: presentations in 12 cases and a review of the literature

INTRODUCCIÓN: La alopecia frontal fibrosante (AFF) es una alopecia cicatricial caracterizada por el retroceso de la línea de implantación del pelo, asociada a alopecia de cejas. Habitualmente afecta a mujeres en edad posmenopáusica, siendo mucho menos prevalente en varones. OBJETIVO: Describir las características clínicas de la AFF en los hombres estudiados y compararlos con los datos recogidos en la literatura. MATERIAL Y MÉTODOS: Se realizó un estudio descriptivo de los varones diagnosticados de AFF en nuestro Servicio, desde enero del 2010 hasta diciembre del 2015. Se recogieron los datos demográficos, las características clínicas y los tratamientos realizados. RESULTADOS: Se reclutó a 12 pacientes. La edad media fue de 75 años. La alopecia fue el motivo de consulta únicamente en 4 pacientes. El retroceso medio de la línea de implantación del pelo fue de 3cm. Las pápulas faciales estaban presentes en el 50% de los hombres, el 83% presentaba alopecia de cejas, extremidades y alopecia androgenética (AGA). El eritema y la hiperqueratosis folicular se veían en el 66% de los casos y solo el 25% refería prurito. El tratamiento más frecuentemente utilizado consistió en corticoide tópico en 8 pacientes (66%), asociado a minoxidil tópico en 4 de ellos (33%). CONCLUSIONES: Según los datos obtenidos en nuestra serie, las pápulas faciales, la AGA y la afectación del vello corporal son más frecuentes en los hombres con AFF que en las mujeres. Por otra parte, a diferencia de los casos de AFF en varones descritos en la literatura, la edad media es mayor en nuestra serie, lo que podría explicar la mayor incidencia de AGA asociada y que la mayoría consulte por otro motivo

BACKGROUND: Frontal fibrosing alopecia (FFA) is a scarring disease in which the hairline recedes and the eyebrows can be affected. Usually seen in postmenopausal women, FFA is much less common in men. OBJECTIVE: To describe the clinical characteristics of FFA in a case series of men and compare this series to those reported in the literature. MATERIAL AND METHODS: Men with FFA being treated in our dermatology department from January 2010 to December 2015 were included prospectively for this descriptive study. We collected patient information and clincal and treatment characteristics. RESULTS: Twelve men (mean age, 75 years) were recruited. Alopecia was the reason for seeking medical care in only 4 cases. The hairline had receded 3cm on average. Half the patients had facial papules, and 83% had androgenetic alopecia or hair loss on eyebrows or extremities. Follicular hyperkeratosis and erythema were present in 66%, and only 25% of the men reported pruritus. The most commonly prescribed treatments were topical: corticosteroids in 8 patients (66%) and minoxidil in 4 (33%). CONCLUSIONS: Facial papules, androgenetic alopecia, and loss of body hair are more often observed in men with FFA than in women. The men in this series were older on average than in other FFA case series in the literature, possibly accounting for the higher prevalence of associated androgenetic alopecia and the fact that most of these men were seeking care for conditions other than hair loss

Frontal Fibrosing Alopecia in Men: Presentations in 12 Cases and a Review of the Literature. / Alopecia frontal fibrosante en el varón: presentación de 12 casos y revisión de la literatura.

BACKGROUND: Frontal fibrosing alopecia (FFA) is a scarring disease in which the hairline recedes and the eyebrows can be affected. Usually seen in postmenopausal women, FFA is much less common in men. OBJECTIVE: To describe the clinical characteristics of FFA in a case series of men and compare this series to those reported in the literature. MATERIAL AND METHODS: Men with FFA being treated in our dermatology department from January 2010 to December 2015 were included prospectively for this descriptive study. We collected patient information and clinical and treatment characteristics. RESULTS: Twelve men (mean age, 75 years) were recruited. Alopecia was the reason for seeking medical care in only 4 cases. The hairline had receded 3cm on average. Half the patients had facial papules, and 83% had androgenetic alopecia or hair loss on eyebrows or extremities. Follicular hyperkeratosis and erythema were present in 66%, and only 25% of the men reported pruritus. The most commonly prescribed treatments were topical: corticosteroids in 8 patients (66%) and minoxidil in 4 (33%). CONCLUSIONS: Facial papules, androgenetic alopecia, and loss of body hair are more often observed in men with FFA than in women. The men in this series were older on average than in other FFA case series in the literature, possibly accounting for the higher prevalence of associated androgenetic alopecia and the fact that most of these men were seeking care for conditions other than hair loss.

Manos de mecánico / Mechanic's hands

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Amylin modulates the formalin-induced tonic pain behaviours in rats.

BACKGROUND: Amylin is a peptide from the calcitonin gene-related peptides (CGRP) family that is expressed by nociceptors. Amylin may modulate pain via a spinal action. METHODS: The effect of amylin's administration on the formalin test of acute and tonic pain was evaluated. Amylin's ability to modulate neuronal activity was analysed by c-Fos expression at the spinal cord lumbar 4-5 region (L4-5) and brain. RESULTS: Amylin subcutaneous administration 20 min prior, but not immediately before formalin, shortened the interphase and anticipated the beginning of the tonic pain phase. Amylin reduced the number of activated spinal cord neurons. Blockade of spinal amylin-receptors by prior L4-5 intrathecal administration of an amylin-receptor antagonist (AC187) attenuated these effects, whereas intrathecal BIBN4096 (CGRP-receptor antagonist) did not, proving that part of amylin's effects were spinally mediated via amylin-receptors. The locus coeruleus and other areas involved in descending modulation and affective responses to pain showed an increased number of activated neurons upon amylin subcutaneous administration, suggesting a role for supraspinal areas in some observed effects. L4-5 intrathecal injection of amylin or AC187 showed that both ligands attenuated tonic pain, but blockade of the amylin-receptor action by AC187 decreased further the number of paw jerks in this period. CONCLUSIONS: Overall, data suggested that amylin modulates pain with an inflammatory component and the autoanalgesic/inhibitory mechanisms occurring in the interphase of the formalin test. Amylin might have affected the nociceptive system at different levels (spinal cord and brain), explaining the different effects observed according to the time of amylin injection. WHAT DOES THIS STUDY ADD?: Amylin modulated formalin interphase and tonic pain behaviours probably by targeting spinal neurons and affecting supraspinal areas involved in affective and modulatory components of pain. Activation of spinal amylin-receptors may contribute to the initiation of inflammatory pain mechanisms.

Larva migrans cutánea de origen autóctono en Guipúzcoa / Autochthonous cutaneous larva migrans infection in Guipúzcoa

INTRODUCCIÓN: Larva migrans cutánea (LM) es una erupción serpiginosa causada por helmintos nematodos que circulan por la epidermis. Se adquiere cuando la piel entra en contacto con tierra contaminada por heces de animales infestados por estos nematodos. Hasta ahora se consideraba como enfermedad importada de zonas tropicales y subtropicales. El objetivo fue estudiar los casos de LM diagnosticados como autóctonos por no haber salido de la provincia de Guipúzcoa recientemente. MATERIAL Y MÉTODOS: Estudio observacional transversal retrospectivo de los casos diagnosticados de LM en el Hospital Universitario Donostia de 2011 a 2015, sin viaje previo a ninguna zona endémica de este cuadro. El diagnóstico fue clínico ante las lesiones características. Las variables estudiadas fueron: edad, género, localización de las lesiones, fecha de inicio de los síntomas, posible fuente de contagio, datos anatomo-patológicos, tratamiento y evolución. RESULTADOS: Se han recogido 4 casos, todos varones, con una media de edad de 60 años. Tres casos presentaron lesiones en las extremidades inferiores, mientras que uno lo hizo en el tronco. Todos nuestros pacientes habían estado en contacto con tierra que pudiera estar contaminada por heces, siendo este el mecanismo de transmisión más probable. Se instauró tratamiento con albendazol oral, con resolución de las lesiones. CONCLUSIONES: La aparición de nuevos casos de LM de origen autóctono en Europa obliga al estudio de la/s especie/s causal/es, a una revisión epidemiológica de esta infestación y a planificar qué medidas se deberían tomar para evitar que una enfermedad considerada hasta ahora como importada, se convierta en autóctona

INTRODUCTION: Cutaneous larva migrans (LM) infection forms a serpiginous eruption caused by the migration of nematode helminths through the epidermis. The parasites are acquired when the skin comes into contact with soil contaminated by the feces of infected animals. Until now, infections have been believed to be imported from tropical and subtropical regions. Our aim was to study cases of cutaneous LM diagnosed in residents of the Spanish province of Guipúzcoa who had not recently traveled to such regions. Material and methods; Cross-sectional observational study of LM cases diagnosed in Hospital Universitario Donostia from 2011 to 2015 in patients who had not visited a region where this nematode infection is endemic. Clinical diagnoses were based on characteristic lesions. We studied the following variables: age, sex, site of lesions, date of onset of symptoms, possible source of contagion, pathologic findings, treatment, and clinical course. RESULTS: We found 4 cases, all in men (mean age, 60 years). Lesions were on the lower extremities in 3 patients and on the trunk in 1 patient. All had been in contact with soil that could have been contaminated by feces and was the most likely source of the parasite. The lesions disappeared after treatment with oral albendazole. CONCLUSIONS: The appearance of cases of autochthonous LM in Europe requires investigation of the culprit species, a review of the epidemiology of this infection, which was once considered imported, and the planning of public health measures to prevent it from becoming endemic

Autochthonous cutaneous larva migrans infection in Guipúzcoa. / Larva migrans cutánea de origen autóctono en Guipúzcoa.

INTRODUCTION: Cutaneous larva migrans (LM) infection forms a serpiginous eruption caused by the migration of nematode helminths through the epidermis. The parasites are acquired when the skin comes into contact with soil contaminated by the feces of infected animals. Until now, infections have been believed to be imported from tropical and subtropical regions. Our aim was to study cases of cutaneous LM diagnosed in residents of the Spanish province of Guipúzcoa who had not recently traveled to such regions. MATERIAL AND METHODS: Cross-sectional observational study of LM cases diagnosed in Hospital Universitario Donostia from 2011 to 2015 in patients who had not visited a region where this nematode infection is endemic. Clinical diagnoses were based on characteristic lesions. We studied the following variables: age, sex, site of lesions, date of onset of symptoms, possible source of contagion, pathologic findings, treatment, and clinical course. RESULTS: We found 4 cases, all in men (mean age, 60 years). Lesions were on the lower extremities in 3 patients and on the trunk in 1 patient. All had been in contact with soil that could have been contaminated by feces and was the most likely source of the parasite. The lesions disappeared after treatment with oral albendazole. CONCLUSIONS: The appearance of cases of autochthonous LM in Europe requires investigation of the culprit species, a review of the epidemiology of this infection, which was once considered imported, and the planning of public health measures to prevent it from becoming endemic.

Tratamiento del síndrome de Sézary con alemtuzumab: serie de 5 casos y revisión de la literatura / Treatment of Sézary Syndrome With Alemtuzumab: A Series of 5 Cases and a Review of the Literature

El alemtuzumab es un anticuerpo monoclonal que se ha utilizado como terapéutica en casos refractarios de síndrome de Sézary (SS) y micosis fungoide en estadio avanzado. Presentamos 5 pacientes diagnosticados de SS tratados con alemtuzumab entre los años 2008 y 2012, con una tasa de respuesta global del 80% (40% respuestas parciales y 40% respuestas completas). La pauta de 10mg vía subcutánea fue bien tolerada y con una toxicidad aceptable. En nuestra casuística la mediana de duración de la respuesta fue de 13 meses, sin embargo uno de los pacientes continúa en remisión completa tras 67 meses, hecho destacable dada la baja supervivencia del SS. Como conclusión, creemos que el alemtuzumab es un fármaco que podría ser útil en casos de SS refractarios a otros tratamientos. Dado que no existen tratamientos curativos en el SS, sería una alternativa terapéutica a tener en cuenta

Alemtuzumab is a monoclonal antibody that has been used to treat refractory cases of Sézary syndrome (SS) and advanced mycosis fungoides. We present 5 patients with SS who were treated with alemtuzumab between 2008 and 2012, with an overall response rate of 80% (40% partial response and 40% complete response). A regimen of 10mg administered subcutaneously was well tolerated with acceptable toxicity. The median duration of response was 13 months. However, one patient remains in complete remission after 67 months, a remarkable outcome given the low survival rate associated with SS. In conclusion, we believe that alemtuzumab may be useful in cases of SS refractory to other treatments. As there are no curative treatments for SS, alemtuzumab should be considered as a therapeutic option

Factores predictores de respuesta y revisión de la toxicidad cutánea de cetuximab y panitumumab en 116 pacientes / Predictors of Tumor Response to Cetuximab and Panitumumab in 116 Patients and a Review of Approaches to Managing Skin Toxicity

INTRODUCCIÓN Y OBJETIVOS: Cetuximab y panitumumab son anticuerpos anti-factor de crecimiento epidérmico (anti-EGFR) usados para el cáncer colorrectal metastásico. La mayoría de los pacientes desarrollan una erupción papulopustulosa que podría predecir la respuesta tumoral. Además, producen otros efectos adversos cutáneos, por lo que hemos estudiado si estos también podrían ser predictores clínicos de respuesta. Así mismo, hemos realizado una revisión del tratamiento de la erupción papulopustulosa, ya que no existen directrices basadas en la evidencia. MATERIAL Y MÉTODOS: Estudio retrospectivo de 116 pacientes. Se incluyeron pacientes afectos de cáncer colorrectal metastásico en tratamiento con los anticuerpos anti-EGFR, cetuximab o panitumumab, en el Hospital Universitario Donostia. RESULTADOS: El 81,9% de los pacientes desarrolló la erupción papulopustulosa, siendo el riesgo mayor y de mayor intensidad cuantos más ciclos de anti-EGFR se administraban (p = 0,03). Todos los pacientes que obtuvieron una respuesta tumoral completa desarrollaron la erupción. Cuanto peor era la respuesta tumoral, menor era la frecuencia de la erupción (p = 0,03). También se encontró una asociación entre la xerosis y la respuesta tumoral (el 53,4% de los que obtuvieron respuesta tumoral desarrollaron xerosis, p = 0,002). El manejo de la erupción papulopustulosa se llevó a cabo mediante un algoritmo desarrollado por nuestro servicio. CONCLUSIONES: En la práctica clínica la erupción papulopustulosa grave y la xerosis pueden ser predictores clínicos de buena respuesta al tratamiento anti-EGFR. Los pacientes con esta erupción deben tratarse precozmente, ya que el tratamiento subóptimo de estos efectos secundarios puede conllevar un retraso en la dosis o su interrupción

INTRODUCTION AND OBJECTIVES: Cetuximab and panitumumab are monoclonal antibodies that target the epidermal growth factor receptor (EGFR) in the treatment of metastatic colorectal cancer. Most patients develop a papulopustular rash, which may predict tumor response. We studied whether the other adverse cutaneous effects associated with these monoclonal antibodies are also clinical predictors of response. We also reviewed publications describing approaches to treating the papulopustular rash since no evidence-based guidelines have yet been published. MATERIAL AND METHODS: We performed a retrospective study of 116 patients with metastatic colorectal cancer receiving anti-EGRF therapy with cetuximab or panitumumab at Hospital Universitario Donostia. RESULTS: In total, 81.9% of the patients developed a papulopustular rash. Patients who received the most cycles of treatment with the EGFR inhibitor were at the highest risk of developing the rash, and these patients also had the most severe rash reactions (P = .03). All of the patients who exhibited a complete tumor response had the rash, and the incidence of rash was lower in patients with poor tumor response (P = .03). We also observed an association between tumor response and xerosis (53.4% of the patients who developed xerosis also exhibited tumor response, P = .002). The papulopustular rash was managed according to an algorithm developed by our department. CONCLUSIONS: Severe papulopustular rash and xerosis may be clinical predictors of good response to anti-EGFR therapy. Patients who develop a papulopustular rash should be treated promptly because suboptimal treatment of this and other adverse effects can lead to delays in taking the prescribed anti-EGFR dose or to interruption of therapy

Treatment of Sézary Syndrome With Alemtuzumab: A Series of 5 Cases and a Review of the Literature.

Alemtuzumab is a monoclonal antibody that has been used to treat refractory cases of Sézary syndrome (SS) and advanced mycosis fungoides. We present 5 patients with SS who were treated with alemtuzumab between 2008 and 2012, with an overall response rate of 80% (40% partial response and 40% complete response). A regimen of 10mg administered subcutaneously was well tolerated with acceptable toxicity. The median duration of response was 13 months. However, one patient remains in complete remission after 67 months, a remarkable outcome given the low survival rate associated with SS. In conclusion, we believe that alemtuzumab may be useful in cases of SS refractory to other treatments. As there are no curative treatments for SS, alemtuzumab should be considered as a therapeutic option.

Predictors of Tumor Response to Cetuximab and Panitumumab in 116 Patients and a Review of Approaches to Managing Skin Toxicity.

INTRODUCTION AND OBJECTIVES: Cetuximab and panitumumab are monoclonal antibodies that target the epidermal growth factor receptor (EGFR) in the treatment of metastatic colorectal cancer. Most patients develop a papulopustular rash, which may predict tumor response. We studied whether the other adverse cutaneous effects associated with these monoclonal antibodies are also clinical predictors of response. We also reviewed publications describing approaches to treating the papulopustular rash since no evidence-based guidelines have yet been published. MATERIAL AND METHODS: We performed a retrospective study of 116 patients with metastatic colorectal cancer receiving anti-EGRF therapy with cetuximab or panitumumab at Hospital Universitario Donostia. RESULTS: In total, 81.9% of the patients developed a papulopustular rash. Patients who received the most cycles of treatment with the EGFR inhibitor were at the highest risk of developing the rash, and these patients also had the most severe rash reactions (P=.03). All of the patients who exhibited a complete tumor response had the rash, and the incidence of rash was lower in patients with poor tumor response (P=.03). We also observed an association between tumor response and xerosis (53.4% of the patients who developed xerosis also exhibited tumor response, P=.002). The papulopustular rash was managed according to an algorithm developed by our department. CONCLUSIONS: Severe papulopustular rash and xerosis may be clinical predictors of good response to anti-EGFR therapy. Patients who develop a papulopustular rash should be treated promptly because suboptimal treatment of this and other adverse effects can lead to delays in taking the prescribed anti-EGFR dose or to interruption of therapy.

Tratamiento con aprepitant del prurito refractario secundario a linfoma cutáneo de células T / Aprepitant in the Treatment of Refractory Pruritus Secondary to Cutaneous T-Cell Lymphoma

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Enfermedad de Grover durante el embarazo / Grover’s disease during pregnancy

Presentamos una mujer embarazada de 37 semanas que consultó por la presencia de una erupción papulovesicular en el tronco de dos semanas de evolución. El examen histopatológico resultó compatible con enfermedad de Grover. Se trata de un caso atípico de una patología relativamente frecuente, en el que se discutimos la posible implicación del embarazo en la patogenia de la dermatosis acantolítica transitoria (AU)

A 37-year-old pregnant woman with a two week s papulovesicular eruption is reported. The histopathological examination showed a Grover s disease. This is an atypical case of a relatively common condition. Herein, we discuss the possible involvement of pregnancy in the pathogenesis of transientacantolitic dermatosis (AU)