[Necrobiosis lipoidica over appendectomy scar in a patient with morphea of the breast]. / Necrobiosis lipoídica sobre cicatriz de apendicectomía en una paciente con morfea de mama.

Morphea is a sclerosing skin disease that may be clinically confused with a carcinoma when it involves the breast. It has been described in association with earlier radiotherapy and with silicone breast implants. We describe the case of a woman with morphea of the breast who developed necrobiosis lipoidica over the scar of an appendectomy performed 38 years earlier. Necrobiosis lipoidica over surgical scars is infrequently found in the literature. We review the published cases and discuss the association of necrobiosis lipoidica with morphea.

Necrobiosis lipoídica sobre cicatriz de apendicectomía en una paciente con morfea de mama / Necrobiosis lipoidica over appendectomy scar in a patient with morphea of the breast

La morfea es una enfermedad esclerosante de la piel que cuando afecta a la mama puede confundirse clínicamente con un carcinoma. Se ha descrito asociada con radioterapia previa y con implantes mamarios de silicona. Se describe el caso de una mujer con morfea de mama que desarrolló una necrobiosis lipoídica sobre una cicatriz de apendicectomía realizada 38 años antes. La necrobiosis lipoídica sobre cicatrices quirúrgicas es un hallazgo poco frecuente en la literatura médica. En este artículo se revisan los casos publicados y se discute la asociación de la necrobiosis lipoídica con la morfea

Morphea is a sclerosing skin disease that may be clinically confused with a carcinoma when it involves the breast. It has been described in association with earlier radiotherapy and with silicone breast implants. We describe the case of a woman with morphea of the breast who developed necrobiosis lipoidica over the scar of an appendectomy performed 38 years earlier. Necrobiosis lipoidica over surgical scars is infrequently found in the literature. We review the published cases and discuss the association of necrobiosis lipoidica with morphea

Tratamiento de linfomas cutáneos de células T con bexaroteno / Treatment of cutaneous T-cell lymphomas with bexarotene

Introducción. La elección del tratamiento en los linfomas cutáneos de células T (LCCT) depende del estadio clínico de la enfermedad y del estado general del paciente. Hasta hoy no existe ningún tratamiento curativo para esta enfermedad y el objetivo es controlar los síntomas y prevenir la progresión de la enfermedad. El bexaroteno es un retinoide agonista específico de los receptores X de los retinoides con actividad antitumoral. Su uso fue aprobado por la Food and Drug Administration (FDA) como tratamiento de LCCT refractarios a al menos una terapia sistémica previa. Pacientes y métodos. Realizamos un estudio descriptivo de 9 pacientes que han sido tratados con bexaroteno en la Unidad de Linfomas de nuestro servicio. Analizamos las características clínicas de los pacientes y eficacia del tratamiento y recogemos los efectos secundarios presentados. Resultados. La respuesta global al tratamiento fue del 44,4 % (4/9). Un total de 2 pacientes presentaron una remisión completa y los otros dos, una remisión parcial. La tolencia al tratamiento fue buena y los efectos secundarios más frecuentes fueron la hipertrigliceridemia, la hipercolesterolemia y el hipotiroidismo central. Conclusiones. Aunque se trata de una serie de únicamente 9 pacientes los resultados que obtuvimos son similares a los descritos previamente. El bexaroteno es una opción terapéutica eficaz en este grupo heterogéneo de enfermedades

Introduction. The choice of treatment in cutaneous T-cell lymphomas (CTCLs) depends on the clinical stage of the disease and the patient's general condition. To date, there is no curative treatment for this disease, and the objective is to control the symptoms and prevent the disease from progressing. Bexarotene is an X receptor-specific retinoid with anti-tumor activity. Its use as treatment for CTCLs refractory to at least one prior systemic therapy has been approved by the FDA. Patients and methods. We carried out a descriptive study of 9 patients treated with bexarotene in the Lymphoma Unit of our department. We analyzed the clinical characteristics of the patients and the efficacy of the treatment, and we collected data on the side effects that appeared. Results. The overall response to the treatment was 44.4 % (4/9). 2 patients had full remission and 2 had partial remission. Tolerance to the treatment was good, and the most frequent side effects were hypertriglyceridemia, hypercholesterolemia and central hypothyroidism. Conclusions. Even though this is a series of only 9 patients, the results that we obtained are similar to ones previously described. Bexarotene is an effective therapeutic option in this heterogeneous group of diseases

Erupción acneiforme por inhibidores del receptor de crecimiento epidérmico / Acneiform eruption from epidermal growth factor receptor inhibitors

Uno de los objetivos fundamentales de la investigación oncológica es la búsqueda de moléculas con mayor eficacia antitumoral y menor toxicidad que los agentes quimioterápicos habituales. Los inhibidores del receptor de crecimiento epidérmico son un nuevo grupo de fármacos que, por su efecto más específico frente a las células neoplásicas, parecen cumplir estas características. Entre los efectos adversos asociados a su uso, destacan por su frecuencia las erupciones cutáneas, secundarias a un efecto inhibitorio directo del fármaco sobre la homeostasis de la epidermis y del folículo pilosebáceo 1,2. Recientemente se han publicado varios casos de toxicidad cutánea en pacientes tratados con inhibidores del receptor de crecimiento epidérmico. Presentamos 3 casos de erupciones acneiformes atribuibles a diferentes fármacos de esta familia (cetuximab, gefitinib y erlotinib)

One of the fundamental aims of oncological research is the search for molecules with greater efficacy against tumors and less toxicity than the usual chemotherapeutic agents. Epidermal growth factor receptor inhibitors are a new group of drugs which, because of their more specific effect against neoplastic cells, seem to meet these characteristics. Skin eruptions are one of the most frequent adverse effects associated with their use, secondary to the drug's direct inhibitory effect on homeostasis of the epidermis and of the pilosebaceous follicle. Several cases of cutaneous toxicity in patients treated with epidermal growth factor receptor inhibitors have recently been published. We present three cases of acneiform eruptions attributable to different drugs in this family (cetuximab, gefitinib and erlotinib)

Allergic contact dermatitis from cyanamide.

Cyanamide (carbimide or calcium carbimide) has been used to treat chronic alcoholism for more than 45 years. The skin reactions provoked by this medication are very rare and are generally associated with systemic reactions after ingestion of the product. However, allergic contact dermatitis has also been detected on persons who prepare cyanamide for administration to patients. We present a case of work-related allergic contact dermatitis from cyanamide that was confirmed by patch testing.

Xantogranuloma múltiple del adulto / Multiple adult xanthogranuloma

El xantogranuloma juvenil es una histiocitosis decélulas no Langerhans (HCNL) poco frecuente, de carácter benigno y que afecta predominantemente a niños pequeños. Clínicamente se caracteriza por la aparición de una o varias lesiones papulonodulares amarillo-parduzcas localizadas en zona superior del cuerpo, sobre todo en cabeza y cuello. Los xantogranulomas en adultos son menos frecuentes y generalmente se presentan como lesiones solitarias. El diagnóstico de xantogranulomas múltiples en adultos es excepcional. Se describe el caso de una paciente diagnosticada de esta rara enfermedad

Juvenile xanthogranuloma is an infrequent, benign,normolipemic, non-Langerhans cell histiocytosis, which primarily affects young children. Clinically, it is characterized by the appearance of one or several brownish-yellow papulonodular lesions on the upper body, especially on the head andn eck. Xanthogranulomas are less frequent in adults, and generally present as solitary lesions. The diagnosis of multiple xanthogranulomasin adults is exceptional. We describe the case of a female patient diagnosed with this rare disease

Urticaria solar. Estudio de 20 casos / Solar urticaria. Study of 20 cases

Introducción. La urticaria solar es una enfermedad poco frecuente, pero probablemente infradiagnosticada. Se caracteriza por la aparición de forma súbita de habones en zonas habitualmente no fotoexpuestas tras irradiación solar o con otras fuentes artificiales de luz visible o ultravioleta. Los casos publicados en la literatura médica son pocos, por lo que la información que tenemos acerca de la enfermedad y su evolución natural es limitada. Material y métodos. Se han recopilado los datos de los 20 pacientes que han sido diagnosticados de urticaria solar en nuestro servicio en los últimos 12 años (1990-2002), para intentar obtener información acerca de las características de esta enfermedad. Resultados. Como características más relevantes de nuestra serie cabe destacar que el 60 % de los pacientes eran mujeres; la duración media de la enfermedad antes de acudir a un dermatólogo era de alrededor de 3 años. En el 55 % de los pacientes se respetaban zonas habitualmente fotoexpuestas como cara y manos (fenómeno de habituación o hardening). Los espectros responsables de la urticaria solar eran luz visible, UVA y UVB, por ese orden. Respecto a los tratamientos, con antihistamínicos y fotoprotectores se obtuvo una respuesta parcial y buenos resultados mediante desensibilizaciones progresivas con UVA/sol. Conclusión. La urticaria solar es probablemente una enfermedad infradiagnosticada en nuestro medio. Hay pocas series publicadas que nos permitan conocer las características más importantes de la enfermedad. Se han recopilado los datos más interesantes de nuestros pacientes y se han comparado con el resto de las series publicadas en el intento de conocer mejor esta fotodermatosis

Introduction. Solar urticaria is an infrequent disorder, but is probably underdiagnosed. It is characterized by the sudden appearance of weals in areas that are not usually photoexposed after exposure to the sun or to artificial sources of visible or ultraviolet light. Few cases have been published in literature, so the information available about this disorder and its natural evolution is limited. Material and methods. We have compiled data from 20 patients diagnosed with solar urticaria in our department in the last 12 years (1990-2002) in order to try to obtain information about the characteristics of this condition. Results. As the most relevant characteristics of our series, we can mention the fact that 60 % of the patients were women, and the average duration of the condition before consulting a dermatologist was 3 years. In 55 % of the patients, areas that are usually photoexposed, such as the face and hands, were less severely affected (due to acclimatization or hardening). The spectra responsible for the SU were visible light, UVA and UVB, in that order. With regard to treatment, we obtained a partial response with antihistamines and photoprotectors, and good results using progressive desensitization with UVA/sunlight. Conclusion. Solar urticaria is probably an underdiagnosed condition in our milieu. There are few series in literature that provide any information about the most significant characteristics of this disorder. We have compiled the most relevant data from our patients and we have compared it with the other published series in an attempt to learn more about this photodermatosis

[Acneiform eruption from epidermal growth factor receptor inhibitors]. / Erupción acneiforme por inhibidores del receptor de crecimiento epidérmico.

One of the fundamental aims of oncological research is the search for molecules with greater efficacy against tumors and less toxicity than the usual chemotherapeutic agents. Epidermal growth factor receptor inhibitors are a new group of drugs which, because of their more specific effect against neoplastic cells, seem to meet these characteristics. Skin eruptions are one of the most frequent adverse effects associated with their use, secondary to the drug's direct inhibitory effect on homeostasis of the epidermis and of the pilosebaceous follicle. Several cases of cutaneous toxicity in patients treated with epidermal growth factor receptor inhibitors have recently been published. We present three cases of acneiform eruptions attributable to different drugs in this family (cetuximab, gefitinib and erlotinib).

[Treatment of cutaneous T-cell lymphomas with bexarotene]. / Tratamiento de linfomas cutáneos de células T con bexaroteno.

INTRODUCTION: The choice of treatment in cutaneous T-cell lymphomas (CTCLs) depends on the clinical stage of the disease and the patient's general condition. To date, there is no curative treatment for this disease, and the objective is to control the symptoms and prevent the disease from progressing. Bexarotene is an X receptor-specific retinoid with anti-tumor activity. Its use as treatment for CTCLs refractory to at least one prior systemic therapy has been approved by the FDA. PATIENTS AND METHODS: We carried out a descriptive study of 9 patients treated with bexarotene in the Lymphoma Unit of our department. We analyzed the clinical characteristics of the patients and the efficacy of the treatment, and we collected data on the side effects that appeared. RESULTS: The overall response to the treatment was 44.4% (4/9). 2 patients had full remission and 2 had partial remission. Tolerance to the treatment was good, and the most frequent side effects were hypertriglyceridemia, hypercholesterolemia and central hypothyroidism. CONCLUSIONS: Even though this is a series of only 9 patients, the results that we obtained are similar to ones previously described. Bexarotene is an effective therapeutic option in this heterogeneous group of diseases.

[Solar urticaria. Study of 20 cases]. / Urticaria solar. Estudio de 20 casos.

INTRODUCTION: Solar urticaria is an infrequent disorder, but is probably underdiagnosed. It is characterized by the sudden appearance of weals in areas that are not usually photoexposed after exposure to the sun or to artificial sources of visible or ultraviolet light. Few cases have been published in literature, so the information available about this disorder and its natural evolution is limited. MATERIAL AND METHODS: We have compiled data from 20 patients diagnosed with solar urticaria in our department in the last 12 years (1990-2002) in order to try to obtain information about the characteristics of this condition. RESULTS: As the most relevant characteristics of our series, we can mention the fact that 60 % of the patients were women, and the average duration of the condition before consulting a dermatologist was 3 years. In 55 % of the patients, areas that are usually photoexposed, such as the face and hands, were less severely affected (due to acclimatization or hardening). The spectra responsible for the SU were visible light, UVA and UVB, in that order. With regard to treatment, we obtained a partial response with antihistamines and photoprotectors, and good results using progressive desensitization with UVA/sunlight. CONCLUSION: Solar urticaria is probably an underdiagnosed condition in our milieu. There are few series in literature that provide any information about the most significant characteristics of this disorder. We have compiled the most relevant data from our patients and we have compared it with the other published series in an attempt to learn more about this photodermatosis.

[Multiple adult xanthogranuloma]. / Xantogranuloma múltiple del adulto.

Juvenile xanthogranuloma is an infrequent, benign, normolipemic, non-Langerhans cell histiocytosis, which primarily affects young children. Clinically, it is characterized by the appearance of one or several brownish-yellow papulonodular lesions on the upper body, especially on the head and neck. Xanthogranulomas are less frequent in adults, and generally present as solitary lesions. The diagnosis of multiple xanthogranulomas in adults is exceptional. We describe the case of a female patient diagnosed with this rare disease.

Neurocristopatía / Neurocristopathy

Las neurocristopatías son las enfermedades secundarias al desarrollo embriológico anómalo de la cresta neural. Al contribuir en la formación de varios tipos de células, las manifestaciones clínicas pueden afectar a una gran variedad de estructuras, entre ellas la piel. Se presenta una niña con un trastorno pigmentario congénito y estudio anatomopatológico compatible con una neurocristopatía (AU)

Dermatitis alérgica de contacto por anillo de madera de cocobolo / Allergic contact dermatitis from a ring made of cocobolo wood

La dermatitis alérgica de contacto en pacientes que utilizan o manipulan objetos de madera acabada en su vida diaria, fuera del ambiente laboral, es excepcional. Las maderas que se han descrito con más frecuencia como responsables de estos casos tan poco frecuentes son, al igual que en los casos ocupacionales, las denominadas "exóticas", procedentes de árboles de países tropicales. Uno de estos árboles es el cocobolo (Dalbergia retusa/obtusa), originario de Centroamérica. Se presenta el caso de una mujer que desarrolló un eccema de contacto en un dedo después de haber llevado un anillo adquirido en un país centroamericano y hecho con madera de cocobolo. El estudio epicutáneo realizado con alergenos específicos de esta madera (obtusaquinona) confirmó la naturaleza alérgica de esta reacción. No hemos encontrado ningún otro caso de dermatitis alérgica de contacto por madera de cocobolo descrito en España (AU)