Complicaciones óseas en un paciente con lepra lepromatosa / Bone Complications in a Patient With Lepromatous Leprosy

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Tricorrexis nodosa localizada / Localized Trichorrhexis Nodosa

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[Clinical experience with efalizumab in the Hospital of Cruces]. / Experiencia clínica con efalizumab en el Hospital de Cruces.

Efalizumab (Raptiva) is one of the biological agents approved recently for the treatment of patients with moderate-severe psoriasis who have not responded to conventional treatments. It is a humanized IgG1 monoclonal antibody which, due to its anti-D11 effect, is capable of blocking the endothelial migration and T cell activation on the skin, fundamental processes in the etiopathogeny of psoriasis. We present the experience we have had in our hospital over the last two years with 23 patients who have initiated treatment with efalizumab.

Coexistencia de morfea en gotas generalizada y liquen escleroatrófico: a propósito de un caso / Patient with generalized guttate morphea and lichen sclerous et atrophicus

La morfea en gotas generalizada es una entidad clínica muy poco frecuente, con escasas referenciasen la literatura. Describimos el caso de un varón de 7 años que acudió por primera vez a nuestra consulta en 1990 presentando este tipo de morfea en el tronco y las extremidades superiores, asociado a la variante en placasen el pie derecho. Doce años después le aparecieron unas lesiones en la región pectoral derecha de aspecto diferente a las previas, y que resultaron ser características clínica e histopatológicamente de liquen escleroso y atrófico (LEA). La morfea y el LEA comparten algunas características clínico-patológicas, y por ello algunos autores creen que podría tratarse de entidades emparentadas, e incluso de dos presentaciones distintas de una misma enfermedad. Creemos que lo más llamativo del caso que describimos es el tipo de morfea que presenta, de la que no hemos encontrado ejemplos equiparables en la literatura (AU)

Generalized guttate morphea is a very uncommon clinical entity, and few reports are available in the literature. We report the case of a 7-year-old boy who first attended our clinic in 1990 with guttate morphea on the trunk and upper limbs. These lesions were associated with plaque morphea on his right foot. Twelve years later, lesions with a different appearance to the previous ones were observed in the right pectoral region. Clinically and histopathologically, they resembled lichen sclerosus et atrophicus. Given that morphea and lichen sclerosus et atrophicus share certain clinical and pathologic characteristics, some authors believe that these entities may be related or even different presentations of the same disease. The most noteworthy aspect of our case is the type of morphea, as we were unable to find equivalent examples in the literature (AU)

Trasplante autólogo de progenitores hematopoyéticos seguido de bexaroteno oral en paciente con micosis fungoide avanzada / Autologous hematopoietic stem cell transplantation folowed by oral bexarotene in a patient with advanced mycosis fungoides

Exponemos el caso de una paciente de 17 años que presentó una micosis fungoide de evolución rápida y agresiva, con múltiples tumores cutáneos y transformación fenotípica celular a linfoma de células grandes. Fue tratada inicialmente con 3 ciclos de quimioterapia en dosis altas (MEGA-CHOP) sin respuesta, por lo que se decidió realizar un trasplante autólogo de progenitores hematopoyéticos. Este tratamiento consiguió una remisión parcial de la enfermedad, y con la posterior introducción del bexaroteno oral se logró la remisión completa, que se ha mantenido durante más de 3 años con muy buena tolerancia al bexaroteno oral. Analizamos las ventajas y desventajas de la utilización del trasplante autólogo de progenitores hematopoyéticos y del uso de bexaroteno oral (AU)

We describe the case of a 17-year-old patient with rapidly progressing and aggressive mycosis fungoides, with multiple cutaneous tumors and large cell transformation. She was initially treated with 3 cycles of high-dose chemotherapy with mega-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) without response, leading to the decision to undertake autologous hematopoietic stem cell transplantation. Partial remission of the disease was achieved with this treatment and subsequent introduction of oral bexarotene led to complete remission, which has been maintained for more than 3 years with good tolerance of oral therapy. We discuss the advantages and disadvantages of autologous hematopoietic stem cell transplantation and the use of oral bexarotene (AU)

[Autologous hematopoietic stem cell transplantation followed by oral bexarotene in a patient with advanced mycosis fungoides]. / Trasplante autólogo de progenitores hematopoyéticos seguido de bexaroteno oral en paciente con micosis fungoide avanzada.

We describe the case of a 17-year-old patient with rapidly progressing and aggressive mycosis fungoides, with multiple cutaneous tumors and large cell transformation. She was initially treated with 3 cycles of high-dose chemotherapy with mega-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) without response, leading to the decision to undertake autologous hematopoietic stem cell transplantation. Partial remission of the disease was achieved with this treatment and subsequent introduction of oral bexarotene led to complete remission, which has been maintained for more than 3 years with good tolerance of oral therapy. We discuss the advantages and disadvantages of autologous hematopoietic stem cell transplantation and the use of oral bexarotene.

[Adalimumab: the molecule and manufacturing procedure]. / Adalimumab: la molécula y el proceso de obtención.

Adalimumab is a fully human monoclonal antibody targeted toward tumor necrosis factor alpha (TNF-alpha). TNF-alpha is proinflammatory cytokine involved in the pathogenesis of many inflammatory diseases, as the psoriasis. The production process of adalimumab is complex and it is based in the called phague display technology.

Adalimumab: la molécula y el proceso de obtención / Adalimumab: the molecule and manufacturing procedure

Adalimumab es un anticuerpo monoclonal totalmente humano y dirigido contra el factor de necrosis tumoral alfa (TNF-α). El TNF-α es una citocina proinflamatoria, implicada en la Etiopatogenia de múltiples entidades, entre ellas la psoriasis. El mecanismo de obtención de adalimumab es complejo y se basa en la denominada técnica de selección mediante presentación en fagos (AU)

Adalimumab is a fully human monoclonal antibody targeted toward tumor necrosis factor alpha (TNF-α). El TNF-α is proinflammatory cytokine envolved in the patogenesis of many inflammatory diseases, as the psoriasis. The production process of adalimumab is complex and it is based in the called phague display technology (AU)

Experiencia clínica con efalizumab en el Hospital de Cruces / Clinical experience with efalizumab in the Hospital of Cruces

Efalizumab (Raptiva®) es uno de los agentes biológicos aprobados recientemente para el tratamiento de pacientes con psoriasis de intensidad moderada-grave que no hayan respondido a tratamientos convencionales. Es un anticuerpo monoclonal humanizado que por su efecto anti-CD11 es capaz de bloquear la migración endotelial y la activación de los linfocitos T a nivel cutáneo, procesos fundamentales en la etiopatogenia de la psoriasis. Presentamos la experiencia que a lo largo de los dos últimos años hemos tenido en nuestro hospital con los 23 pacientes que han iniciado tratamiento con efalizumab (AU)

Efalizumab (Raptiva®) is one of the biological agents approved recently for the treatment of patients with moderate-severe psoriasis who have not responded to conventional treatments. It is a humanized IgG1monoclonal antibody which, due to its anti-D11 effect, is capable of blocking the endothelial migration and Tcell activation on the skin, fundamental processes in the etiopathogeny of psoriasis. We present the experience we have had in our hospital over the last two years with 23 patients who have initiated treatment with efalizumab (AU)

[Eruptive pseudoangiomatosis: study of 7 cases]. / Pseudoangiomatosis eruptiva: 7 casos clínicos.

INTRODUCTION: Eruptive pseudoangiomatosis consists in the acute development of small vascular lesions in the face and extremities that resolve in several weeks without scarring. Lesions are described as 3-4 mm asymptomatic macules and papules with peripheral whitish halo that blanch upon pressure. Initially it was considered a disease limited to children but it has also been described in adults. It overlaps with the entity known in Japan as <>, possibly caused by an insect named Culex pipiens pallens. METHODS: We report a serie of 7 patients that consulted for lesions compatible with eruptive pseudoangiomatosis. We performed a detailed clinical history and histological, microbiological and serological studies. Follow-up time was up to 4 years. RESULTS: Eighty-five percent of patients were women and the mean age was 62 years. All cases appeared in spring/summer and 71 % relapsed. Lesions predominated in the face and extremities and the outbreak lasted 2-4 weeks. The anamnesis did not disclose any specific etiologic agent in any of the cases. Complete laboratory tests including serologies and cultures were negative or within normal limits. Histological study revealed vascular dilatation in all cases with endothelial cell protrusion and a peripheral lymphohistiocytic infiltrate. CONCLUSIONS: Currently, the etiology of this entity is not well established although it probably represents a reactive disorder to different etiologic processes.

Pseudoangiomatosis eruptiva: 7 casos clínicos / Eruptive pseudoangiomatosis: study of 7 cases

Introducción. La pseudoangiomatosis eruptiva consiste en la aparición aguda de lesiones de aspecto angiomatoso de pequeño tamaño en cara y extremidades y su desaparición en el curso de varias semanas sin dejar cicatriz residual. Las lesiones se describen como máculo-pápulas de 3-4 mm, con un halo blanquecino periférico, asintomáticas y que blanquean a la vitropresión. En un principio se pensaba que era un cuadro limitado a niños, pero posteriormente se ha descrito también en adultos. Se considera superponible a un cuadro conocido en Japón como erythema punctatum Higuchi y posiblemente causado por un insecto denominado Culex pipiens pallens. Métodos. Presentamos una serie de 7 pacientes que consultaron en nuestro Servicio por un cuadro compatible con la pseudoangiomatosis eruptiva. Se les realizó una historia clínica detallada, estudio histológico, microbiológico y serológico. El seguimiento fue de hasta 4 años. Resultados. El 85 % de los pacientes fueron mujeres, y la edad media fue de 62 años. Todos los casos se iniciaron en los meses de primavera/verano y el 71 % sufrieron recidivas. Las localizaciones predominantes fueron la cara y las extremidades y la duración del brote fue de 2 a 4 semanas. La historia clínica no permitió establecer un agente desencadenante en ninguno de los casos. Las analíticas, estudios histológicos, serologías y cultivos estuvieron dentro de los rangos de normalidad. En las imágenes histológicas apreciamos en todos los casos dilatación vascular, con cierta protrusión de las células endoteliales hacia la luz del vaso y un infiltrado de predominio linfohistiocitario periférico. Conclusiones. Hoy en día la etiología de este cuadro clínico sigue sin estar bien establecida, aunque probablemente suponga una manifestación clínico-patológica reactiva a distintos procesos etiológicos

Introduction. Eruptive pseudoangiomatosis consists in the acute development of small vascular lesions in the face and extremities that resolve in several weeks without scarring. Lesions are described as 3-4 mm asymptomatic macules and papules with peripheral whitish halo that blanch upon pressure. Initially it was considered a disease limited to children but it has also been described in adults. It overlaps with the entity known in Japan as «erythema punctatum Higuchi», possibly caused by an insect named Culex pipiens pallens. Methods. We report a serie of 7 patients that consulted for lesions compatible with eruptive pseudoangiomatosis. We performed a detailed clinical history and histological, microbiological and serological studies. Follow-up time was up to 4 years. Results. Eighty-five percent of patients were women and the mean age was 62 years. All cases appeared in spring/summer and 71 % relapsed. Lesions predominated in the face and extremities and the outbreak lasted 2-4 weeks. The anamnesis did not disclose any specific etiologic agent in any of the cases. Complete laboratory tests including serologies and cultures were negative or within normal limits. Histological study revealed vascular dilatation in all cases with endothelial cell protrusion and a peripheral lymphohistiocytic infiltrate. Conclusions. Currently, the etiology of this entity is not well established although it probably represents a reactive disorder to different etiologic processes