Certolizumab pegol effectiveness, survival and safety in patients with psoriasis: A multicenter retrospective analysis in daily clinical practice by the Spanish Psoriasis Group.

BACKGROUND: Certolizumab is an Fc-free PEGylated tumor necrosis factor-alpha (TNFα) inhibitor recently approved for the treatment of moderate-to-severe plaque psoriasis, although there is limited real-world evidence on the effectiveness and safety in patients with plaque psoriasis treated with certolizumab. The objective of this article is to determine the effectiveness, drug survival, and safety, including pregnancy, childbirth, and lactation, of certolizumab in moderate-to-severe plaque psoriasis under real-world conditions. METHODS: This is a retrospective, multicenter, observational study performed in 15 hospitals in Spain. It evaluates the effectiveness and safety of certolizumab in plaque psoriasis in the clinical practice setting. RESULTS: A total of 67 patients (73% female) were evaluated with a mean baseline Psoriasis Area Severity Index (PASI) of 8.9. At Week 12, the mean PASI was 2.3 (n = 67), 1.3 (n = 57) at Week 24 and 1.3 at Week 52 (n = 34). Absolute PASI < 3 was achieved in 69, 86, and 92% of patients at Weeks 12, 24, and 52, respectively, as observed. For its part, using the under-response imputation analysis, PASI < 3 at Weeks 12, 24, and 52 were achieved by 69, 73, and 49% of the patients, respectively. A total of 35 patients (52%) had concomitant psoriatic arthritis, and, in 24 of them, Disease Activity in Psoriatic Arthritis Score (DAPSA) was recorded at baseline, with a mean value of 17.9 which decreased to 8.2 at Week 12 (n = 22) and to 3.6 at Week 24 (n = 18). Certolizumab treatment was discontinued in 14 out of 67 patients (21%), due to lack/loss of cutaneous or articular effectiveness (n = 11) or patient decision (n = 2) or adverse event in only one patient who developed active tuberculosis. A lower baseline PASI [hazard ratio (HR): 1.12 (1.02-1.23); P = 0.023] and a more significant reduction in PASI at Week 12 [HR: 1.16 (1.07-1.27); P < 0.001] and Week 52 [HR: 1.47 (1.11-1.96); P = 0.007] was shown to be significantly related with better survival for the entire follow-up period. Fourteen patients were treated during pregnancy and/or lactation without reporting adverse events in either the patient or the newborn. CONCLUSIONS: Certolizumab consistently showed high effectiveness and drug survival rates in this real-life cohort. The safety demonstrated in clinical trials during pregnancy and lactation seems to be confirmed in clinical practice.

Hidradenitis suppurativa of the nape: Description of an atypical phenotype related to severe early-onset disease in men.

Some patients with hidradenitis suppurativa (HS) develop severe inflammatory lesions of the nape. Through a single-center, cross-sectional study with a total of 377 patients, we sought to compare patients with and without nape involvement, to determine whether disease severity is greater in these patients and to describe their clinical characteristics. Thirty patients (90% male) were identified as having nape involvement. Patients with versus without nape involvement had greater disease severity, earlier disease onset, a family history of HS, lower body mass, and higher Dermatological Life Quality Index and pain scores. Amongst them, involvement of the trunk and gluteal regions predominated. A significant univariate relationship existed between patients with nape involvement and male sex, early disease onset, family history, scalp and gluteal involvement, Canoui-Poitrine phenotypes II and III, and Dowling-Degos disease. A significant multivariate relationship existed with early disease onset, Dowling-Degos disease, and phenotypes II and III.

Circulating levels of lipocalin-2 and retinol-binding protein-4 are increased in psoriatic patients and correlated with baseline PASI.

Psoriasis has been related to metabolic syndrome (MS). Adipocytokines produced by white adipose tissue may be involved in the pathogenesis of psoriasis and its association with MS. Our objectives were to characterize the profile of a number of different inflammatory and atherogenic markers, vitamins, adipokines and cytokines and their potential involvement in MS in patients with moderate-to-severe psoriasis without joint involvement compared to anthropometrically matched controls, and to evaluate correlation with severity of the skin disease and changes after narrow-band UVB (NB-UVB) phototherapy. We designed a prospective cross-sectional study. Baseline waist circumference, body fat composition, lipid, carbohydrate and calcium metabolism profile, inflammation markers, homocysteine and vitamins D, B6, B12 and folic acid, leptin, resistin, omentin, lipocalin-2, adipocyte fatty acid-binding protein, retinol-binding protein-4 (RBP-4), interleukin-6, soluble tumour necrosis factor receptor 1 (sTNFR1) and interleukin-17 of 50 psoriasis patients and 50 gender, age and body mass index-matched controls were recorded, then evaluated after NB-UVB in the patients. The patients had higher baseline serum concentrations of leptin, RBP-4, lipocalin-2 and sTNFR1. Baseline psoriasis area and severity index correlated with serum concentrations of RBP-4 and lipocalin-2 only. Principal components analysis disclosed a component including vitamins B12, B6, folic acid, calcidiol and HDL-cholesterol that was only present in healthy controls and opposed to a cluster of variables which promote MS. This component was absent in the patients. Our results point to lipocalin-2 and RBP-4 as relevant mediators of the trend towards MS in psoriatic patients.

Acute dermal abscesses caused by Serratia marcescens.

Primary acute cutaneous infections caused by Serratia marcescens are extremely unusual. Nevertheless, Serratia infections are especially frequent in chronic granulomatous disease, which is a primary immunodeficiency that affects phagocytic cells of the innate immune system. We report a young man without history of infections, who developed multiple dermal abscesses on a leg with chronic lymphoedema attributed to S marcescens. Laboratory investigations showed a delayed partial neutrophilic oxidative function. It is remarkable that the patient did not have any other infections during childhood, when most of the innate immune deficiencies are diagnosed, and he had no history of granulomatous lesions. We hypothesize that the delayed neutrophilic oxidative function could be explained by a partial neutrophilic oxidative function, which could be enough to maintain the patient asymptomatic until this infection.

Narrow elliptical skin excision and cartilage shaving for treatment of chondrodermatitis nodularis.

BACKGROUND: Surgical excision remains the hallmark of therapy for chondrodermatitis nodularis chronica (CNC). Although excision is generally successful, recurrence at the edge of the excised nodule is well recognized. OBJECTIVE: To analyze the clinical features and the recurrence rate of patients with CNC treated with elliptical excision of the papule and removal of the underlying cartilage. DESIGN: Seventy-four outpatients with CNC, 52 men and 22 women, were retrospectively analyzed. A narrow elliptical excision of the papule followed by a slice of the underlying cartilage were carried out with local anesthetic. Cartilage spikes were trimmed carefully shaping a smooth contour from the normal helical rim to the defect. RESULTS: Good cosmetic results were obtained in all patients. The median follow-up for helical and antihelical lesions were 54 and 50 months, respectively. The global recurrence rate was 13.5% and ranged from 10.6 to 37.5% for helical and antihelical lesions, respectively. CONCLUSIONS: We recommend this surgical procedure because it has been shown to be as efficient as other methods and probably faster to perform.

Relationships between lung function, smoking and morphology of dermal elastic fibres.

OBJECTIVE: To investigate the relationship between lung function and dermal elastic fibres in non-smokers and smokers with and without chronic obstructive pulmonary disease (COPD). METHOD: A cross-sectional study was performed with lung function as the outcome [forced expiratory volume in the first second (FEV1)%, FEV1/forced vital capacity (FVC)% and residual volume (RV)/total lung capacity (TLC)%] and smoking (pack-years) and morphology of dermal elastic fibres (fibres/mm(2) and percentage histologic field filled with them) as independent variables, with assessment of the associations between these variables by univariate and multivariate testing. RESULTS: Sixty outpatients reporting chronic cough and/or phle\gm were enrolled (16 non-smokers/44 smokers; COPD: 26, 43.3%). Both lung function and elastic fibres in the reticular dermis (fibres/mm(2)r(s) = 0.36, percentage of the histologic field filled by elastic fibres r(s) = 0.48, P < or = 0.01) correlated with cumulative smoking (Spearman's rank correlation coefficient). Lung function parameters correlated with percentage of the histologic field filled by elastic fibres (FEV1%: r = -0.43; FEV1/FVC%: r = -0.36 and RV/TLC%: r = 0.43; P < or = 0.01, Pearson correlation coefficient), and COPD was significantly related to this morphologic parameter (odds ratio 1.26, 95% confidence interval 1.06-1.48; P = 0.006), associations that depended mainly on cumulative smoking. CONCLUSION: Lung function impairment with an obstructive pattern is associated with morphologic abnormalities in the reticular dermis appearing in the histologic section as an increase in the percentage of the field filled by elastic fibres. This relationship depends on cumulative smoking and suggests a common effect of smoking on the elastic fibres in both lung and skin.

Alteraciones de las fibras elásticas de la piel en pacientes fumadores y su relación con el deterioro de la función pulmonar / Alterations in the elastic fibers of the skin in smokers and their relation to the deterioration of pulmonary function

Objetivo: Investigar los efectos del consumo de tabaco sobre las fibras elásticas de la dermis, así como la relación entre estas fibras elásticas de la dermis y la función pulmonar, tanto en no fumadores como en fumadores. Método: Estudio transversal con los parámetros de función pulmonar (volumen espiratorio forzado en el primer segundo, FEV1/capacidad vital forzada, FVC) como variables dependientes y el consumo de tabaco y la morfología de las fibras elásticas de la dermis, analizada mediante morfometría estática (número y densidad de las fibras elásticas de la dermis en piel no fotoexpuesta), como variables independientes. La asociación entre las variables se analizó mediante tests bivariados y multivariados. Resultados: Se incluyeron 60 pacientes en el estudio (media 58,1; desviación estándar [DE] 6,9 años/16 no fumadores, 26 ex fumadores y 18 fumadores activos). La morfometría de la dermis reticular mostró un aumento del número y de la densidad de las fibras elásticas en fumadores (666,6 DE 208,7 fibras/mm2 y 145,4 DE 3,8%) respecto a los no fumadores (506,5 DE 156,1 fibras/mm2 y 11,3 DE 1,7%) (p < 0,01, test t). Los parámetros de función pulmonar mostraron una correlación con la densidad de las fibras elásticas (FEV1: r = -0,43; FVC: r = -0,31; coeficiente de correlación de Pearson). La asociación entre la densidad de las fibras elásticas y FEV1 (coeficiente de correlación parcial = -0,27; p = 0,04) se mantuvo estadísticamente significativa después de ajustar por el consumo de tabaco. Conclusión: Los pacientes fumadores presentan un aumento significativo de la densidad de las fibras elásticas de la piel respecto a los no fumadores. Asimismo, este aumento de la densidad de las fibras elásticas de la dermis muestra una correlación con el deterioro de la función pulmonar. Esta relación depende sólo parcialmente del consumo de tabaco, hallazgo que sugiere la existencia de factores genéticos que afectarían de forma similar a las fibras elásticas del pulmón y a las de la piel (AU)

Erupción cutánea en la primoinfección por el virus de la inmunodeficiencia humana / Skin eruption in primary human immunodeficiency virus infection

La primoinfección por el virus de la inmunodeficiencia humana (VIH) es sintomática en el 50-90 por ciento de los casos, a pesar de lo cual, en la mayoría de ellos, el diagnóstico pasa desapercibido. El conjunto de manifestaciones clínicas se conoce como síndrome retroviral agudo, el cual está relacionado con el alto índice de replicación del retrovirus y la intensa respuesta del sistema inmunitario frente a éste. Los síntomas más frecuentes son fiebre, fatiga, erupción cutánea y linfadenopatías generalizadas, que suelen aparecer entre la 2.ª y la 4.ª semanas de la infección. La erupción suele afectar la cara y el tercio superior del tronco, y muestra máculas y pápulas eritematosas anaranjadas, redondeadas, no confluentes, algunas con necrosis central. En muchas ocasiones se acompaña de afectación de las mucosas. Se describen 4 casos de primoinfección por el VIH. El diagnóstico en esta fase de la enfermedad se establece mediante técnicas de laboratorio no serológicas (carga viral, ADN proviral) y permite instaurar el tratamiento antirretroviral de forma precoz (AU)

A case of multiple keratoacanthoma centrifugum marginatum.

BACKGROUND: Keratoacanthoma centrifugum marginatum is a rare variety of keratoacanthoma, usually presented and classified as solitary keratoacanthoma. Reported cases of multiple keratoacanthoma centrifugum marginatum are exceptional in the literature. OBSERVATIONS: A 21-year-old man presented for evaluation of erythematous papules and plaques on both legs that had had a peripheral growth over the past year. Clinical and histologic examination showed typical features of keratoacanthoma centrifugum marginatum. Owing to the multiplicity and size of the lesions, treatment with oral isotretinoin was started. It was ineffective. Subsequent treatment with methotrexate combined with oral prednisone led to the regression of all lesions. CONCLUSIONS: The exceptional case of a man with multiple keratoacanthoma centrifugum marginatum is reported. Treatment with methotrexate combined with oral prednisone was effective. This case illustrates how some variants of keratoacanthomas do not really fit into the current classification and how complex the treatment of these tumors is.

Dermatosis eosinofílica asociada a leucemia linfática crónica: estudio clínico, microscópico e inmunohistoquímico de 5 casos / Eosinophilic dermatosis associated with chronic lymphocytic leukemia: clinical, microscopic and immunohistochemical study of five cases

Introducción. En los pacientes con leucemia linfática crónica (LLC) se ha descrito un grupo de dermatosis caracterizadas por la eosinofilia tisular que se ha denominado reacción similar a picadura o, más recientemente, dermatosis eosinofílica de los síndromes mieloproliferativos. Pacientes y métodos. Se recogen 5 pacientes diagnosticados de LLC que padecieron una erupción papulovesiculosa característica, parecida a las picaduras de insecto. Se realizó una biopsia en sacabocados en los 5 pacientes para el estudio histológico e inmunohistoquímico con anticuerpos monoclonales CD3, CD5 (células T), CD20, CD79a y CD23 (células B).Resultados. Tres mujeres y dos varones con edades comprendidas entre los 50 y 85 años desarrollaron entre una y 30 lesiones en forma de pápulas o placas eritematovioláceas en el tronco y las extremidades. Tres de los pacientes presentaron además febrícula y síndrome constitucional. El estudio histológico de las biopsias mostraba un infiltrado en la dermis superficial y profunda compuesto por linfocitos, polimorfonucleares y abundantes eosinófilos. La mayor parte de las células mononucleares fueron positivas para los marcadores de linfocitos T, y fueron negativas para células B. Tres de los 5 pacientes sufrieron progresión a un estadio más avanzado de LLC. Conclusión. La dermatosis eosinofílica en pacientes con una LLC constituye una entidad reactiva y característica que a menudo precede a una progresión de la enfermedad de base. Por el momento se desconoce su patogenia exacta y su posible implicación pronóstica (AU)

Carcinoma epidermoide sobre lupus vulgar / Squamous cell carcinoma over lupus vulgaris

La tuberculosis cutánea es una infección poco frecuente en España. Su forma clínica de presentación más común es el lupus vulgar. Es conocido que pueden desarrollarse carcinomas epidermoides sobre úlceras y lesiones cutáneas inflamatorias de larga evolución, entre ellas el lupus vulgar, sobre todo si han sido tratados previamente con radioterapia. Se presentan 2 casos de carcinoma epidermoide desarrollado sobre lesiones de lupus vulgar de más de 40 años de evolución, localizadas en la región pectoral en el primer caso y en la cara en el segundo. Ninguno de ellos había recibido ningún tratamiento antes del diagnóstico del carcinoma epidermoide. La prueba de la tuberculina fue fuertemente positiva en ambos casos, pero sólo en uno de ellos pudo aislarse Mycobacterium tuberculosis en el cultivo de las lesiones cutáneas. Cabe destacar que en nuestros casos la radioterapia no ha intervenido como factor inductor en el desarrollo de la neoplasia (AU)

Granuloma anular macular. Estudio de cinco casos / Patch granuloma annulare. Report of five cases

Introducción. El granuloma anular es una dermatosis de gran variabilidad clínica. Una variante de reciente descripción es la denominada macular. Se describen 5 casos de esta entidad y se efectúa una revisión bibliográfica. Pacientes y método. Se incluyeron en el estudio 5 pacientes que desarrollaron lesiones cutáneas compatibles con un granuloma anular de la variedad macular. En todos ellos se realizó una biopsia "en sacabocados" y se revisaron las características epidemiológicas, clínicas y anatomopatológicas. Resultados. Se trataba de 4 mujeres y un varón, con edades comprendidas entre los 58 y 66 años. Todos ellos presentaban lesiones de meses de evolución localizadas en los muslos, excepto en un caso, que presentaba las lesiones en los antebrazos. Se trataba de máculas redondeadas eritematovioláceas o parduscas de 5 a 15 cm de diámetro y sin cambios epidérmicos. Tres pacientes tenían una única lesión y los otros dos, varias de distribución bilateral y simétrica. En el estudio histológico se observó en todos los casos un infiltrado linfohistiocitario intersticial con necrobiosis difusa y depósitos de mucina. Conclusión. El granuloma anular macular se caracteriza, en la clínica, por la aparición de lesiones maculares eritematosas de crecimiento centrífugo. En la microscopia, el infiltrado linfohistiocitario y la necrobiosis se disponen de una forma difusa, siguiendo un patrón intersticial. El diagnóstico de esta variante de granuloma anular es difícil si no existe un alto índice de sospecha por parte del clínico y del patólogo (AU)

Antinuclear autoantibodies, complement level, hypergammaglobulinemia and spontaneous intrauterine hematoma in pregnant women.

PROBLEM: To examine the associative relationship among autoantibodies, C4 levels and intrauterine hematomas (IUH) in more detail than in the studies published earlier. METHOD OF STUDY: We performed a retrospective study of 54 women with poor obstetric outcomes. Sera were screened for antinuclear antibodies (ANA), anti-DNA antibodies, antiphospholipid antibodies (aPL), and antithyroid antibodies. C4-complement and gammaglobulin levels were also monitored. We compared the main variables in IUH complicated pregnancy group with the risk pregnancy group without IUH. We also compared these variables in the IUH cases before and during IUH. RESULTS: Eight IUH were detected. The average number of spontaneous losses for these eight women was 3.3 +/- 2.1 (range: 1-8). aPL was present in 100% of cases. ANAs and hypergammaglobulinemia were present in 50% of cases and low C4 in 87.5% of cases. After comparing these variables apart from C4 before and during IUH, we found no statistical differences. However, C4 was low in four patients before IUH and in seven patients during IUH (OR: 7.0; 95% CI: 0.57-86.33). When we compared autoantibodies apart from lupus anticoagulant (LAC) between the two groups, no differences were observed. However, seven of the eight (87.5%) patients with IUH were LAC positive whereas only 24 of the 46 patients (52.1%) were positive in the non-IUH group (OR: 6.42; 95% CI: 0.73-56.41). Rapid plasma reagin was present in 8/46 in the non-IUH group (16.7%) and 5/8 in the IUH group (62.5%) P < 0.015). CONCLUSIONS: In women with poor obstetric histories, autoantibodies, especially antiphospholid antibodies, may play a role in the IUH development especially if low C4 and/or hypergammaglobulinemia are present.

The ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC): report of five cases.

We report five patients from two different pedigrees with the ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC). All had features of ectodermal dysplasia, but only three had limb ectrodactyly and orofacial clefting. The present patients illustrate the great phenotypic variability in the EEC syndrome. As no single feature, including any of the three cardinal signs, is mandatory for syndrome diagnosis, a meticulous examination of all family members is needed.

Surgical management of rhinophyma: report of eight patients treated with electrosection.

BACKGROUND: Rhinophyma is the end stage of rosacea, affecting almost exclusively male patients, characterized by a progressive deformity in nasal shape. Depending on the extent of the deformity, rhinophyma may distort the appearance of those affected and patients may become psychosocially disabled. OBJECTIVE: To present the results of electrosurgical technique of sequential delamination with a slightly damped current in rhinophyma. METHODS: Eight healthy male patients affected by moderate to severe rhinophyma were recruited. A radiofrequency electrosurgical unit was used in the cut/coagulate mode at 40 W (3.8 MHz) to remove thin layers of tissue until the nose shape was re-created. RESULTS: All patients achieved acceptable cosmetic results. Cutaneous reepithelialization was achieved at day 14 after surgery. Erythema subsided within 4-8 weeks and healing was complete with minimal scar formation by 8-10 weeks. None of the patients developed hypertrophic scarring and hypopigmentation was minimal. CONCLUSION: Electrosection is as effective as laser treatments and less expensive. Although living in a laser era, electrosurgical remodeling of the nose is a good alternative for the treatment of rhinophyma.

Erupción acneiforme a tipo Halogenodermia por Hidantoínas / Acneiform eruption with Phenytoin Halogenoderma expression

Las halogenodermias son dermatosis poco frecuentes que aparecen tras la ingesta o inhalación de sustancias o fármacos que contienen haluros, generalmente, yoduros y bromuros. Describimos un caso de erupción acneiforme a tipo halogenodermia en un paciente que recibía tratamiento anticonvulsionante con fenitoína (AU)

Pustulosis aguda generalizada / Pustulosis acuta generalisata

El término pustulosis aguda generalizada define una erupción pustulosa, estéril, aguda y autolimitada de distribución acral y simétrica desencadenada por una infección faríngea estreptocócica. Otros procesos pueden expresarse en la piel con una clínica similar, siendo el diagnóstico diferencial entre ellos, en muchas ocasiones, difícil. Creemos que la mayoría de ellos pueden corresponder a un mismo patrón de reacción de la piel frente a determinados estímulos, normalmente un fármaco o una infección. Así podrían incluirse dentro del espectro de las dermatosis neutrofílicas como una única entidad bajo el término de pustulosis reactivas. Se presentan tres casos que ilustran las dificultades diagnósticas de la pustulosis aguda generalizada y su clasificación nosológica (AU)

Lipoatrofia semicircular de los muslos / Semicircular lipoatrophy of the thighs

La lipodistrofia semicircular de los muslos es una entidad infrecuente que se caracteriza por la atrofia localizada del tejido celular subcutáneo en áreas expuestas a microtraumatismos repetidos. Aportamos los casos de dos mujeres de 32 y 38 años en las que el factor precipitante más probable fue el traumatismo provocado por el uso de mallas deportivas (AU)