Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients.

INTRODUCTION: PRP is a rare entity of unknown etiopathogenesis. Lack of management guidelines makes it a challenge for clinicians. OBJECTIVE: To add our experience to increase evidence about PRP. METHODS: We performed a retrospective, descriptive and multicentric study of 65 patients with PRP, being the largest European case series of patients with PRP. RESULTS: PRP was more frequent in male patients with an average age of 51 years, but erythrodermic forms presented in older patients (average age 61 years). Six (75%) paediatric patients and ten (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. On the contrary, 26 (68%) erythrodermic patients required biologic therapy as last and effective therapy line requiring an average of 6.5 months to achieve complete response. CONCLUSION: Our study showed a statistical difference in terms of outcome and response to treatment between children or patients with limited disease and patients who develop erythroderma.

Subclinical enthesopathy of extensor digitorum tendon is highly prevalent and associated with clinical and ultrasound alterations of the adjacent fingernails in patients with psoriatic disease.

BACKGROUND: Nail psoriasis disease is associated with an increased probability of psoriatic arthritis, and its clinical signs may have different correlates with the pathogenesis of adjacent bone destruction and have different prognostic value. Recent publications about psoriasis and nail psoriatic disease describe different ultrasonographic findings but the relationship between these ungueal alterations measured by ultrasonography and the presence of enthesopathy of the extensor digitorum has yet to be discovered. OBJECTIVE: To describe which ultrasonographic characteristics of nail psoriasis are associated with the presence of subclinical enthesopathy in patients with PsO and asymptomatic PsA. METHODS: Patients with psoriasis and asymptomatic psoriatic arthritis were included in the prospective study. Demographic, clinical data and PASI and NAPSI indexes were recorded of all the patients in the assessment visit. The US assessment included Achilles tendon, extensor digitorum tendon and US scan of the nail plate, nail matrix, nail bed and adjacent skin over nail matrix of the five nails of each hand. RESULTS: Forty-eight patients were included in the study; 33 of them presented ultrasound evidence of extensor digitorum tendon enthesopathy. Nails of the patients with subclinical enthesopathy had a higher NAPSI and skin thickness than the nails of the patients without subclinical enthesopathy (P = 0.047). Patients with asymptomatic enthesopathy had significantly thicker proximal nail folds (1.44 ± 0.312 vs. 1.23 ± 0.27, P = 0.023). Nail beds and matrices were also thicker but the differences were not statistically significant (1.77 ± 0.27 vs. 1.74 ± 0.21, P = 0.66, and 1.79 ± 0.28 vs. 1.67 ± 0.19, P = 0.10, respectively). No statistically significant differences in the trilaminar structure were found between both groups. Patients with and without asymptomatic enthesopathy of extensor digitorum tendons did not statistically differ as regards ultrasonographic alterations of the Achilles tendons (60.6% vs. 46.4%, P 0.368). CONCLUSION: Enthesopathy abnormalities can be detected by US in patients with psoriasis without musculoskeletal complaints frequently. There is a close relationship between subclinical enthesopathy of the extensor digitorum tendon and the presence of nail alterations. Further studies are required to research what implications have the presence of these ungual alterations measured by US, and how it affects later development of a PsA.

Infantile hemangiomas with minimal or arrested growth associated with soft tissue hypertrophy: a case series of 10 patients.

BACKGROUND: Infantile hemangiomas with minimal or arrested growth (IH-MAGs) are characterized by a proliferative component of <25% of its surface area. The co-occurrence of IH-MAGs and soft tissue anomalies is rare, and case series of this association are lacking. OBJECTIVE: We present 10 cases of IH-MAGs associated with soft tissue hypertrophy and describe their clinical features. METHODS: We reviewed all infantile hemangiomas with minimal or arrested growth seen between 2009 and 2016 in the dermatology clinic department at Hospital Santa Creu i Sant Pau, Barcelona. To collect more patients, we also requested cases from the Hemangioma Investigator Group and members of the Spanish Society of Vascular Anomalies. RESULTS: Ten patients had IH-MAGs associated with soft tissue hypertrophy; seven involving the arm and three involving the leg. All displayed a segmental pattern, a doughy and puffy texture and prominent surface veins. No significant asymmetries in limbs and no other visceral anomalies were observed at follow-up (range 15 months to 7 years). One patient reported coldness in the limb with infantile hemangioma, but RMI-angiography did not disclose a vascular malformation underneath the lesion. Ulceration was observed in three patients. The proliferative component in all IH-MAGs had faded at 1-year follow-up, while soft tissue hypertrophy and prominent vessels remained unchanged. CONCLUSIONS: In this first case series of IH-MAGS associated with soft tissue hypertrophy, soft tissue hypertrophy was not progressive and remained unchanged over time, unlike the proliferative component of classic infantile hemangioma. The origin of the prominent vessels and the higher ulceration risk are unknown; however, these findings are probably related to a minor disruption of local vessels not detected in imaging tests.

Ictiosis en bañador y diagnóstico prenatal en subsiguiente embarazo / Prenatal Screening for Bathing-suit Ichthyosis After Diagnosis in an Older Sibling

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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

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.

[Feasibility and reliability of the Spanish version of the Leeds Revised Acne Grading Scale]. / Factibilidad y fiabilidad de la versión española de la escala revisada de gravedad de Leeds (LRAG) para pacientes con acné.

BACKGROUND: Although there are more than 25 acne grading systems, there is no consensus on which is most appropriate. Unification of the classifications is recommended in order to facilitate therapeutic decisions. OBJECTIVE: To assess the feasibility and reliability of the Spanish version of the Leeds revised acne grading (LRAG) scale in patients with acne vulgaris in Spain. PATIENTS AND METHODS: We conducted a prospective, multicenter, observational study in Spain, including patients with acne affecting at least 1 of 3 regions: face, back, or chest. Patients were assessed using the LRAG scale and lesion counting. Changes in the scores were determined at 4-6 weeks, and were correlated with the lesion count. Physicians were asked 4 questions regarding difficulty using the scale and the time employed. RESULTS: A total of 259 sites of acne were assessed in 239 patients at 57 centers. The majority of physicians (89.5%) stated that the LRAG scale was not difficult to use. The mean administration time was 3.12min. Cross-sectional validity (P<.012 for the face, P<.001 for the back and chest), longitudinal validity (P<.0001 for the face, back, and chest), and intraobserver and interobserver reliability (Cronbach α >0.8) were significant for inflammatory lesions in all regions. Sensitivity to change was demonstrated for lesions in all regions, based on the correlation between the difference in severity and the number of lesions recorded by the LRAG, and the difference in the lesion count between baseline and follow-up. CONCLUSION: The Spanish version of the LRAG scale is a practical and reliable tool and is sensitive to change. It is a valid tool for the objective assessment of the severity of acne.

Factibilidad y fiabilidad de la versión española de la escala revisada de gravedad de Leeds (LRAG) para pacientes con acné / Feasibility and Reliability of the Spanish Version of the Leeds Revised Acne Grading Scale

Introducción: Se describen más de 25 métodos para valorar la gravedad del acné y, aunque no hay consenso sobre una escala, se recomienda unificar su clasificación para facilitar las decisiones terapéuticas. Objetivos: Validar la factibilidad y fiabilidad de la escala revisada de gravedad de Leeds (LRAG) en pacientes con acné vulgar en España. Material y métodos: Estudio observacional prospectivo multicéntrico español que evaluó mediante la escala LRAG y el recuento de lesiones a pacientes con acné en al menos una de tres localizaciones (cara, pecho o espalda). Se analizaron los cambios en la puntuación a las 5±1 semanas y se correlacionaron con el recuento de lesiones. El clínico respondió 4 preguntas sobre dificultad y tiempo de uso de la escala. Resultados: Fueron evaluadas 259 localizaciones de acné en 239 pacientes y en 57 centros asistenciales. El 89,5% [IC: 85–92,9%] de los médicos opinaron que la escala se usó sin dificultad y su tiempo medio de administración fue de 3,12min. La validez transversal (p<0,012 facial, p<0,001 espalda y pecho), validez longitudinal (p<0,0001) y fiabilidad intra e interobservador (alfa de Cronbach ≥0,8) de la escala fue significativa en todas las localizaciones. En relación a sensibilidad al cambio de la escala, las lesiones observadas en todas las localizaciones en el seguimiento dependen de la diferencia de gravedad registrada por la escala LRAG y el recuento de lesiones basales. Conclusiones: La versión española de la escala LRAG es factible, fiable, sensible y constituye una herramienta válida para objetivar clínicamente la gravedad del acné (AU)

Background: Although there are more than 25 acne grading systems, there is no consensus on which is most appropriate. Unification of the classifications is recommended in order to facilitate therapeutic decisions. Objective: To assess the feasibility and reliability of the Spanish version of the Leeds revised acne grading (LRAG) scale in patients with acne vulgaris in Spain. Patients and methods: We conducted a prospective, multicenter, observational study in Spain, including patients with acne affecting at least 1 of 3 regions: face, back, or chest. Patients were assessed using the LRAG scale and lesion counting. Changes in the scores were determined at 4–6 weeks, and were correlated with the lesion count. Physicians were asked 4 questions regarding difficulty using the scale and the time employed. Results: A total of 259 sites of acne were assessed in 239 patients at 57 centers. The majority of physicians (89.5%) stated that the LRAG scale was not difficult to use. The mean administration time was 3.12 min. Cross-sectional validity (P<0.012 for the face, P<0.001 for the back and chest), longitudinal validity (P<.0001 for the face, back, and chest), and intraobserver and interobserver reliability (Cronbach α >0.8) were significant for inflammatory lesions in all regions. Sensitivity to change was demonstrated for lesions in all regions, based on the correlation between the difference in severity and the number of lesions recorded by the LRAG, and the difference in the lesion count between baseline and follow-up. Conclusion: The Spanish version of the LRAG scale is a practical and reliable tool and is sensitive to change. It is a valid tool for the objective assessment of the severity of acne (AU)

[Psoriasis, a systemic disease?]. / La psoriasis, una enfermedad sistémica?

It has long been recognized the epidemiological association of psoriasis, especially the most severe forms, with several diseases that share a common pathogenic substrate involving TNF-alpha and different target organs (arthritis and Crohn's disease, for example), as well as an increased risk of coronary heart disease and occlusive cardiovascular disease. In the patient with severe psoriasis there is also an increased prevalence of obesity, dyslipemia, adult diabetes mellitus, alcohol abuse and tobacco habit which contribute to the increased risk of mortality associated with atherosclerosis. Recently it has been identified the so-called metabolic syndrome, characterized by the association of abdominal obesity, atherogenic dyslipemia, hypertension, insulin resistance with or without glucose intolerance and a proinflammatory and prothrombotic state as a risk factor for cardiovascular disease. There is evidence that in rheumatoid arthritis as well as in psoriasis, chronic inflammation has a pathogenic role in the metabolic syndrome and associated comorbidities, and its adequate treatment may contribute to revert it. The dermatologist should recognize the elements of the metabolic syndrome and propose the patient with psoriasis, in addition to the optimal dermatologic treatment, changes in life habits and appropriate drug therapy to reduce the risk of cardiovascular morbi-mortality.

La psoriasis, ¿una enfermedad sistémica? / Psoriasis, a systemic disease?

Desde hace tiempo se conoce la asociación epidemiológica de la psoriasis, especialmente en sus formas graves, con diversas enfermedades con las que comparte un sustrato patogénico común, con implicación del factor de necrosis tumoral alfa (TNF-alfa) y diferente órgano diana (tales como artritis y enfermedad de Crohn, por ejemplo), así como con un aumento en el riesgo de cardiopatía coronaria y enfermedad cardiovascular oclusiva. En el paciente con psoriasis grave también se encuentra un aumento en la prevalencia de obesidad, dislipemia, diabetes mellitus del adulto, enolismo y tabaquismo, que contribuyen al incremento en el riesgo de mortalidad relacionada con la aterosclerosis. Recientemente se ha identificado el denominado síndrome metabólico, caracterizado por la asociación de obesidad abdominal, dislipemia aterogénica, hipertensión, resistencia a la insulina con o sin intolerancia a la glucosa y un estado proinflamatorio y protrombótico como factor de riesgo de enfermedad cardiovascular, y existen evidencias de que, tanto en la artritis reumatoide como en la psoriasis, la inflamación crónica tiene un papel patogénico en el síndrome metabólico y las comorbilidades asociadas, y su tratamiento adecuado podría contribuir a revertirlo. Constituye una obligación del dermatólogo reconocer los elementos del síndrome metabólico y proponer al paciente con psoriasis, además del tratamiento óptimo de la psoriasis, los cambios de hábitos de vida y el tratamiento farmacológico adecuado para reducir el riesgo de morbimortalidad cardiovascular

It has long been recognized the epidemiological association of psoriasis, especially the most severe forms, with several diseases that share a common pathogenic substrate involving TNF-alpha and different target organs (arthritis and Crohn's disease, for example), as well as an increased risk of coronary heart disease and occlusive cardiovascular disease. In the patient with severe psoriasis there is also an increased prevalence of obesity, dyslipemia, adult diabetes mellitus, alcohol abuse and tobacco habit which contribute to the increased risk of mortality associated with atherosclerosis. Recently it has been identified the so-called metabolic syndrome, characterized by the association of abdominal obesity, atherogenic dyslipemia, hypertension, insulin resistance with or without glucose intolerance and a proinflammatory and prothrombotic state as a risk factor for cardiovascular disease. There is evidence that in rheumatoid arthritis as well as in psoriasis, chronic inflammation has a pathogenic role in the metabolic syndrome and associated comorbidities, and its adequate treatment may contribute to revert it. The dermatologist should recognize the elements of the metabolic syndrome and propose the patient with psoriasis, in addition to the optimal dermatologic treatment, changes in life habits and appropriate drug therapy to reduce the risk of cardiovascular morbi-mortality