Protein S deficiency revealed by skin necrosis in a patient with lupus.

Protein S deficiency is rare in systemic lupus erythematosus (SLE) and is generally associated with the presence of antiphospholipid (APL) antibodies. Lack of protein S can cause skin necrosis, but when it does it is generally in response to warfarin exposure. In this article, we describe the case of a patient who had not received warfarin and without APL antibodies who developed extensive skin necrosis due to protein S deficiency. It is important to investigate protein S deficiency in patients with lupus and extensive skin ulcers as it is a sign of arterial thrombosis and venous thromboembolism.

Lesiones orales y coriorretinitis placoide posterior como forma de presentación de sífilis secundaria en un paciente inmunocompetente / Secondary syphilis presenting as oral lesions and posterior placoid chorioretinitis in an immunocompetent patient

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Milia en placa en la cara posterior de ambos pabellones auriculares secundaria a radioterapia / Milia en plaque on the posterior surface of both auricules following radiation therapy

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Toxicodermias por telaprevir en el tratamiento de la infección crónica por el genotipo 1 del virus de la hepatitis C. Estudio prospectivo / Drug eruptions induced by telaprevir in patients with chronic hepatitis C virus genotype 1 infection: a prospective study

INTRODUCCIÓN: Telaprevir es un fármaco que administrado junto a interferón y ribavirina incrementa de forma significativa la respuesta al tratamiento de la infección por el virus de la hepatitis C. Sin embargo, su empleo incrementa también la probabilidad de desarrollar efectos adversos, en muchos casos cutáneos que pueden condicionar el mantenimiento del tratamiento. OBJETIVO: Conocer la incidencia, características clínicas y evolutivas y respuesta al tratamiento de las toxicodermias por telaprevir en el contexto del tratamiento de la infección por el virus de la hepatitis C. MATERIAL Y MÉTODOS: Estudio prospectivo observacional realizado entre mayo de 2012 y julio de 2013 en el que se incluyeron aquellos pacientes que iniciaron tratamiento con telaprevir durante ese periodo. En aquellos en los que se detectaron toxicodermia se recogieron los datos demográficos de los pacientes, las características clínicas de las lesiones y la evolución tras la aplicación de las recomendaciones de las guías clínicas. RESULTADOS: De un total de 43 pacientes que recibieron tratamiento triple un 46% presentó toxicodermia atribuible a telaprevir. En el 90% de los casos esta fue leve o moderada (grados 1 o 2) y consistió en un exantema constituido por pápulas y placas eritematoedematosas y descamativas. En alrededor de un tercio de los pacientes se comprobó la progresión de la toxicodermia, principalmente en extensión, durante el curso del tratamiento. En 2 casos (4,6%) las lesiones cutáneas condicionaron la suspensión del fármaco. Un 79% de los tratados (34 pacientes) alcanzó una respuesta viral sostenida tras el tratamiento. CONCLUSIONES: Las toxicodermias asociadas a telaprevir son frecuentes en el curso del tratamiento y a menudo progresivas. Sin embargo, solo de forma excepcional condicionan su suspensión

INTRODUCTION: When co-administered with interferon and ribavirin, the prescription drug telaprevir significantly improves treatment response in patients with chronic hepatitis C virus (HCV) infection. Its use, however, also increases the likelihood of adverse effects that may lead to discontinuation of treatment. Cutaneous adverse effects are particularly common. OBJECTIVE: To determine the frequency and clinical characteristics of drug eruptions induced by telaprevir in patients receiving HCV treatment and to analyze the clinical course of lesions and response to treatment. MATERIAL AND METHODS: We performed a prospective observational study of all patients who started a treatment regimen that included telaprevir between May 2012 and July 2013. We recorded the demographic characteristics of the patients who developed telaprevir-induced eruptions, and analyzed the clinical characteristics of the lesions and their clinical course following the application of guideline-based treatment recommendations. RESULTS: Twenty (46%) of the 43 patients who received triple therapy with interferon, ribavirin, and telaprevir during the study period developed drug reactions attributable to telaprevir. The reaction was classified as mild or moderate (grades 1 or 2) in 90% of cases and consisted of an exanthem with erythematous-edematous scaling plaques and papules. The rash worsened, mainly by spreading, in about one-third of cases. The skin lesions led to discontinuation of treatment in 2 patients (4.6%). Sustained viral response was achieved in 34 patients (79%). CONCLUSIONS: Telaprevir-induced eruptions are common and often progress, but they rarely require patients to discontinue treatment

Drug eruptions induced by telaprevir in patients with chronic hepatitis C virus genotype 1 infection: a prospective study.

INTRODUCTION: When co-administered with interferon and ribavirin, the prescription drug telaprevir significantly improves treatment response in patients with chronic hepatitis C virus (HCV) infection. Its use, however, also increases the likelihood of adverse effects that may lead to discontinuation of treatment. Cutaneous adverse effects are particularly common. OBJECTIVE: To determine the frequency and clinical characteristics of drug eruptions induced by telaprevir in patients receiving HCV treatment and to analyze the clinical course of lesions and response to treatment. MATERIAL AND METHODS: We performed a prospective observational study of all patients who started a treatment regimen that included telaprevir between May 2012 and July 2013. We recorded the demographic characteristics of the patients who developed telaprevir-induced eruptions, and analyzed the clinical characteristics of the lesions and their clinical course following the application of guideline-based treatment recommendations. RESULTS: Twenty (46%) of the 43 patients who received triple therapy with interferon, ribavirin, and telaprevir during the study period developed drug reactions attributable to telaprevir. The reaction was classified as mild or moderate (grades 1 or 2) in 90% of cases and consisted of an exanthem with erythematous-edematous scaling plaques and papules. The rash worsened, mainly by spreading, in about one-third of cases. The skin lesions led to discontinuation of treatment in 2 patients (4.6%). Sustained viral response was achieved in 34 patients (79%). CONCLUSIONS: Telaprevir-induced eruptions are common and often progress, but they rarely require patients to discontinue treatment.

Directrices del grupo español de psoriasis (GEP) basadas en la evidencia para el uso de medicamentos biológicos en pacientes con psoriasis en localizaciones de difícil tratamiento (uñas, cuero cabelludo, palmas y plantas) / Evidence-Based Guidelines of the Spanish Psoriasis Group on the Use of Biologic Therapy in Patients With Psoriasis in Difficult-to-Treat Sites (Nails, Scalp, Palms, and Soles)

El término de psoriasis en localizaciones de difícil tratamiento se emplea para hacer referencia a la psoriasis localizada en el cuero cabelludo, las uñas, las palmas y las plantas y que requiere un manejo diferenciado. A menudo los pacientes presentan un importante impacto físico y emocional, unido a la dificultad para controlar adecuadamente sus lesiones con tratamientos tópicos, debido a una insuficiente penetración de los principios activos y la escasa cosmeticidad de los vehículos empleados. Esta circunstancia justifica que la psoriasis en estas localizaciones pueda ser considerada grave, a pesar de su extensión limitada. La experiencia con terapias biológicas en estas localizaciones es escasa, en general en el contexto de ensayos clínicos de formas extensas de psoriasis moderada y grave, junto con series limitadas o casos aislados. En el presente artículo se presenta la calidad de la evidencia científica para los 4 agentes biológicos disponibles en España (infliximab, etanercept, adalimumab y ustekinumab) siendo de nivel i en el caso de la psoriasis ungueal (nivel de recomendación A) y algo inferior en la psoriasis del cuero cabelludo y palmoplantar

Psoriatic lesions affecting the scalp, nails, palms, and the soles of the feet are described as difficult-to-treat psoriasis and require specific management. Involvement of these sites often has a significant physical and emotional impact on the patient and the lesions are difficult to control with topical treatments owing to inadequate penetration of active ingredients and the poor cosmetic characteristics of the vehicles used. Consequently, when difficult-to-treat sites are involved, psoriasis can be considered severe even though the lesions are not extensive. Scant information is available about the use of biologic therapy in this setting, and published data generally comes from clinical trials of patients who also had moderate to severe extensive lesions or from small case series and isolated case reports. In this article we review the quality of the scientific evidence for the 4 biologic agents currently available in Spain (infliximab, etanercept, adalimumab, and ustekinumab) and report level i evidence for the use of biologics to treat nail psoriasis (level of recommendation A) and a somewhat lower level of evidence in the case of scalp involvement and palmoplantar psoriasis

Distrofia fascial congénita o síndrome de la piel rígida: presentación de un caso / Congenital Fascial Dystrophy or Stiff Skin Syndrome: A Case Report

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Evidence-based guidelines of the spanish psoriasis group on the use of biologic therapy in patients with psoriasis in difficult-to-treat sites (nails, scalp, palms, and soles).

Psoriatic lesions affecting the scalp, nails, palms, and the soles of the feet are described as difficult-to-treat psoriasis and require specific management. Involvement of these sites often has a significant physical and emotional impact on the patient and the lesions are difficult to control with topical treatments owing to inadequate penetration of active ingredients and the poor cosmetic characteristics of the vehicles used. Consequently, when difficult-to-treat sites are involved, psoriasis can be considered severe even though the lesions are not extensive. Scant information is available about the use of biologic therapy in this setting, and published data generally comes from clinical trials of patients who also had moderate to severe extensive lesions or from small case series and isolated case reports. In this article we review the quality of the scientific evidence for the 4 biologic agents currently available in Spain (infliximab, etanercept, adalimumab, and ustekinumab) and report level i evidence for the use of biologics to treat nail psoriasis (level of recommendation A) and a somewhat lower level of evidence in the case of scalp involvement and palmoplantar psoriasis.

Paquidermodactilia tratada con éxito con infiltraciones de triamcinolona / Pachydermodactyly Successfully Treated With Triamcinolone Injections

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Acroqueratodermia acuagénica siríngea / Aquagenic syringeal acrokeratoderma

La acroqueratodermia acuagénica siríngea es una entidad poco frecuente. Consiste en una maceración de la piel de las palmas, similar a la que ocurretras el contacto prolongado con el agua, pero que se manifiesta a los pocos minutos de exposición y se resuelve también de forma rápida tras susecado. La etiopatogenia es desconocida y su histopatología variable. No obstante, la clínica muy característica, hace fácil su diagnóstico si se conocela entidad. El tratamiento no está estandarizado pero el uso de cloruro de aluminio hexahidratado ha mostrado buena respuesta en algunos casos (AU)

Aquagenic syringeal acrokeratoderma is a rare entity characterized by the palmar maceration similar to that usually seen after a long exposition towater. Nevertheless, this condition is manifested after a short contact with water and it also resolves rapidly after drying the hands. Despite its unclearorigin and variable histopathology, once it is known, it is easy to diagnose. The treatment is not standardized, although good responses with aluminiumchloride hexahidrate have been reported (AU)

Enfermedad por émbolos de colesterol: estudio de 16 casos / Cholesterol embolism disease: report of 16 cases

Fundamento. La enfermedad por émbolos de colesterol (EEC) es un proceso multisistémico producido por la embolización de microcristales de colesterol procedentes de los grandes vasos en los órganos periféricos, fundamentalmente la piel, el riñón y el sistema nervioso central (SNC). Las intervenciones intravasculares y los tratamientos anticoagulantes suelen ser los factores desencadenantes. Pacientes y métodos. Se registraron de forma prospectiva los datos clínicos, analíticos y anatomopatológicos de 16 pacientes diagnosticados de EEC en nuestro centro entre 1991 y 1998. Resultados. La media de edad fue de 68 años. Todos los pacientes presentaban factores de riesgo y manifestaciones clínicas preexistentes de aterosclerosis avanzada. Los factores desencadenantes identificados con más frecuencia fueron los tratamientos anticoagulantes con heparina en siete pacientes y cumarínicos en cuatro, seguidos de las maniobras angioinvasivas diagnósticas y la cirugía vascular en siete pacientes. Las manifestaciones cutáneas estuvieron presentes en todos los pacientes y fueron: livedo reticular (12), dedos de pies purpúricos (12), lesiones purpúricas, úlceras dolorosas y gangrena. La biopsia cutánea realizada fue diagnóstica en diez pacientes, siendo altamente sugestiva en el resto. Once pacientes desarrollaron insuficiencia renal, aunque sólo cinco precisaron diálisis. Dos pacientes presentaron clínica neurológica y tres digestiva. Sólo dos pacientes fallecieron como consecuencia de complicaciones de la enfermedad por émbolos de colesterol. Conclusiones. El diagnóstico de enfermedad por émbolos de colesterol debe considerarse en todos los pacientes con antecedentes de aterosclerosis avanzada que han sido sometidos a un agente precipitante y desarrollan hipertensión y/o fallo renal acompañado de las típicas lesiones cutáneas. Dadas las graves complicaciones a las que puede conducir esta enfermedad y la falta de un tratamiento eficaz es preciso extremar las medidas preventivas en los pacientes de riesgo. (AU)

[Cholesterol embolism disease: study of 16 cases]. / Enfermedad por émbolos de colesterol: estudio de 16 casos.

BACKGROUND: The cholesterol embolism syndrome is a multisystemic disease resulting from cholesterol crystal embolization to many organs including skin, kidney and CNS. Vascular procedures and anticoagulation have been identified as triggering factors. PATIENTS AND METHODS: Sixteen patients were prospectively reviewed diagnosed of cholesterol embolism syndrome from 1991 to 1998. RESULTS: The mean age was 68 years and all had at least two risk factors for atherosclerosis (hypertension, smoking, diabetes mellitus, hyperlipemia) as well as pre-existing symptomatic atherosclerotic disease. At least one precipitating factor was identified in 14 patients (heparin in 7, coumarins in 4 and vascular procedure in 7). In six patients two or more triggering factors coexisted. Clinically, 12 patients had livedo reticularis, 10 purpuric lesions, 12 purple toes and 4 painful ulcerations. As a result of progressive gangrene 4 patients required amputation of a portion of the lower extremity. The skin biopsy was diagnostic of cholesterol embolism syndrome in 10 cases and was highly suggestive in the remaining cases. Eleven patients developed renal failure but only five required subsequent dialysis. A cerebrovascular accident was reported in two patients and gastrointestinal bleeding occurred in another three patients. Four patients died but only two as a direct result of the disease. CONCLUSIONS: The diagnosis of cholesterol embolisms should be considered among elderly patients, with underlying atherosclerotic disease, who develop typical cutaneous manifestations, hypertension, and renal failure in association with precipitating factors. Given the serious implications of this syndrome, a heightened awareness and preventive measures in the population at risk are essential.

[Classical Kaposi sarcoma of aggressive course]. / Sarcoma de Kaposi clásico de comportamiento agresivo.

The aggressive variant of the classical Kaposi sarcoma (KS), though uncommon, has an utmost relevance because its evolutive characteristics entail a great morbidity and mortality. Three classical KS cases are reported which manifested an unusually aggressive course, with extensive cutaneous and visceral involvement. One of the patients was diagnosed of a non-Hodgkin lymphoma 12 months later. None of the three patients responded to the different therapeutic regimens used: polychemotherapy, monochemotherapy and interferon-alfa, respectively. The three patients died within two years after diagnosis. In KS patients with pulmonary involvement, working in coal mines was recorded as occupational antecedent. These three cases illustrate the different clinical and therapeutic characteristics of an uncommon subtype in the KS spectrum in general and of its classical variant in particular. Likewise, the possible role of occupational exposure in the etiopathogenesis of KS is discussed.

[Transient neonatal pustular melanosis]. / Melanosis pustular transitoria neonatal.

Ten newborn infants with transient neonatal pustular melanosis are reported. The incidence (0.59%), the most noticeable clinical aspects and the differential diagnosis are pointed out. We report with special emphasis the presence of eosinophils in the smears from skin lesions. The authors think that transient neonatal pustular melanosis and erythema toxicum neonatorum are, probably, two different clinical expressions of the same entity.