Trastornos autoinflamatorios de la queratinización / Autoinflammatory Keratinitzation Diseases

La enfermedad autoinflamatoria de la queratización (AiKD, por sus siglas en inglés) es un concepto clínico novedoso que engloba a las enfermedades que presentan antecedentes genéticos, así como mecanismos patogénicos mixtos de autoinflamación y autoinmunidad, lo que en su conjunto se traducirá en una queratinización aberrante de la piel. Los recientes avances han revelado causas genéticas y/o factores predisponentes para una serie de AiKD, dentro de los que se pueden enumerar la presencia de las mutaciones en el IL36RN, en relación con la psoriasis pustulosa, la acrodermatitis continua y la hidradenitis supurativa; en el CARD14, en relación con la pitiriasis rubra pilaris tipo V y algunas formas de psoriasis pustulosa, y en el NLRP1 en relación con la queratosis liquenoide crónica familiar (KLC, por sus siglas en inglés). Se sospecha que la fisiopatología de la AiKD también estaría presente en algunos trastornos no monogénicos. Se debe de comprender que existe una relación bidireccional entre la inflamación y la queratinización para poder determinar un tratamiento óptimo; así mismo para poder desarrollar nuevos fármacos ambos factores deben de tenerse en cuenta. Probablemente en los próximos años nuevas enfermedades inflamatorias de la queratinización serán incluidas dentro del grupo de las AiKD (AU)

Autoinflammatory keratinization disease (AiKD) is a novel clinical concept encompassing diseases with a genetic background and mixed pathogenic mechanisms of autoinflammation and autoimmunity, leading to an aberrant keratinization of the skin. Recent advances in medical genetics have revealed genetic causes and/or predisposing factors for a number of AiKD's, such as mutations in IL36RN related with pustular psoriasis, acrodermatitis continua and hidradenitis suppurativa, in CARD14 in pityriasis rubra pilaris type V and some forms of pustular psoriasis, and in NLRP1 related with familial keratosis lichenoides chronica (KLC). It is suspected that AiKD pathophysiology would also be involved in non-monogenic disorders. The bidirectional relationship between inflammation and keratinization should be understood in order to outline optimal management, and new drug development should take both targets into account. We assume that new inflammatory keratinization diseases may be recognized as AiKDs in the coming years (AU)

Autoinflammatory keratinitzation diseases (AIKDs. / Trastornos autoinflamatorios de la queratinización.

Autoinflammatory keratinization disease (AiKD) is a novel clinical concept encompassing diseases with a genetic background and mixed pathogenic mechanisms of autoinflammation and autoimmunity, leading to an aberrant keratinization of the skin. Recent advances in medical genetics have revealed genetic causes and/or predisposing factors for a number of AiKD's, such as mutations in IL36RN related with pustular psoriasis, acrodermatitis continua and hidradenitis suppurativa, in CARD14 in pityriasis rubra pilaris type V and some forms of pustular psoriasis, and in NLRP1 related with familial keratosis lichenoides chronica (KLC). It is suspected that AiKD pathophysiology would also be involved in non-monogenic disorders. The bidirectional relationship between inflammation and keratinization should be understood in order to outline optimal management, and new drug development should take both targets into account. We assume that new inflammatory keratinization diseases may be recognized as AiKDs in the coming years.

Algoritmo de tratamiento con omalizumab en urticaria crónica espontánea / Algorithm for Treatment of Chronic Spontaneous Urticaria with Omalizumab

Antecedentes y objetivo: Los ensayos pivotales de omalizumab en urticaria crónica espontánea (UCE) tienen un periodo de tratamiento de entre 12 y 24 semanas. Sin embargo, muchos pacientes en práctica clínica requieren periodos de tratamiento más prolongados. Por ello el objetivo es presentar un algoritmo de manejo del fármaco. Materiales y métodos: El documento de consenso que detallamos nace de la puesta en común, aceptación, revisión y confrontación de la literatura reciente del grupo de trabajo de UCE "Xarxa d'Urticària Catalana i Balear" (XUrCB). Resultados: Se inicia el tratamiento a dosis autorizada y se ajusta la dosis en intervalos trimestrales en función del Urticaria Activity Score de los últimos 7 días (UAS7) y/o el Urticarial Control Test (UCT). Conclusiones: El algoritmo propuesto pretende servir de guía respecto a cómo ajustar dosis, cómo y cuándo parar el fármaco y el modo de reintroducirlo en casos de recaída

Background and objective: Pivotal trials with omalizumab for treatment of chronic spontaneous urticaria (CSU) are generally run over 12 to 24weeks. However, in clinical practice, many patients need longer treatment. In this article, we present an algorithm for treatment with omalizumab. Material and methods: The consensus document we present is the result of a series of meetings by the CSU working group of "Xarxa d'Urticària Catalana i Balear" (XUrCB) at which data from the recent literature were presented, discussed, compared, and agreed upon. Results: Treatment with omalizumab should be initiated at the authorized dose, and is adjusted at 3-monthly intervals according to the Urticaria Activity Score Over 7 days, the Urticaria Control Test, or both. Conclusions: The algorithm proposed is designed to provide guidance on how to adjust omalizumab doses, how and when to discontinue the drug, and how to reintroduce it in cases of relapse

Algorithm for Treatment of Chronic Spontaneous Urticaria with Omalizumab. / Algoritmo de tratamiento con omalizumab en urticaria crónica espontánea.

BACKGROUND AND OBJECTIVE: Pivotal trials with omalizumab for treatment of chronic spontaneous urticaria (CSU) are generally run over 12 to 24weeks. However, in clinical practice, many patients need longer treatment. In this article, we present an algorithm for treatment with omalizumab. MATERIAL AND METHODS: The consensus document we present is the result of a series of meetings by the CSU working group of "Xarxa d'Urticària Catalana i Balear" (XUrCB) at which data from the recent literature were presented, discussed, compared, and agreed upon. RESULTS: Treatment with omalizumab should be initiated at the authorized dose, and is adjusted at 3-monthly intervals according to the Urticaria Activity Score Over 7days, the Urticaria Control Test, or both. CONCLUSIONS: The algorithm proposed is designed to provide guidance on how to adjust omalizumab doses, how and when to discontinue the drug, and how to reintroduce it in cases of relapse.

Dermanyssus gallinae (chicken mite): an underdiagnosed environmental infestation.

Dermanyssus gallinae is a mite that normally parasitizes small birds but may occasionally bite humans. We report an unusual case of an 82-year-old woman who presented with pruritus and bite-like lesions over her trunk. Other members of the household were also affected. On physical examination, mites < 1 mm in size were found on the patient's body. The family were residing in the city centre and had no pets, but there were pigeon nests in close proximity to the house. Most dermatologists have difficulties identifying ectoparasitosis. In the case of D. gallinae, the small size of the mites and the fact that they leave the host after feeding means that they may not be seen at presentation, thus such infestations are likely to be underdiagnosed. Physicians should be aware that infection with this mite is possible even in patients from urban areas, and it should be included in the differential diagnosis of conditions causing recurrent pruritus unresponsive to standard treatments.

Psoriasis ungueal: tratamiento con ungüento hidrófilo de tazaroteno al 0.1 por ciento / Nail Psoriasis: Treatment With Tazarotene 0.1 percent Hydrophilic Ointment

Introducción: La afectación ungueal de la psoriasis es una presentación frecuente que interfiere de manera significativa en la calidad de vida de los pacientes. Su presentación clínica va a depender del área ungueal afecta: lecho o matriz. Un 50% de los pacientes refiere dolor asociado. En este estudio evaluamos la eficacia y seguridad del tazaroteno 0,1% en ungüento hidrófilo. Material y métodos: Estudio abierto y observacional de 6 pacientes diagnosticados de psoriasis ungueal. Se aplicó ungüento de tazaroteno 0,1% (fórmula magistral) en oclusión nocturna, en su domicilio durante 6 meses, sin otro tratamiento tópico o sistémico. Se determinó el Nail Psoriasis Severity Index (NAPSI) y se evaluaron la hiperqueratosis subungueal, onicólisis, hemorragias en astilla, manchas de aceite y piquiteado ungueal, en la visita basal, a los 3 y 6 meses. Resultados: Se observó una mejoría estadísticamente significativa en todos los pacientes: NAPSI basal, media ± DE 14,3± 6,3; IC 95% 11,74-16,92; mediana 15; NAPSI a los 6 meses: media ± DE 2,3 ±1,21; IC 95% 1,84-2,83; mediana 2,5; p=0,007. El porcentaje de mejoría fue del 87,9% al final del tratamiento. No se registraron efectos adversos. Conclusión: Nuestro estudio muestra un potencial terapéutico del ungüento de tazaroteno en la psoriasis ungueal (AU)

Introduction: Nail involvement is common in psoriasis and has a considerable impact on patient quality of life. Its clinical presentation depends on which part of the nail is affected: the bed or the matrix. Fifty percent of patients report associated pain. In this study, we analyzed the safety and effectiveness of tazarotene 0.1% in a hydrophilic ointment in the treatment of nail psoriasis. Material and methods: We performed an open observational study of 6 patients diagnosed with nail psoriasis. The patients applied a compounded preparation of tazarotene 0.1% ointment under occlusion every night for 6 months in their homes. They were not receiving any other topical or systemic treatments. Nail psoriasis severity (assessed using the Nail Psoriasis Severity Index [NAPSI]), subungual hyperkeratosis, onycholysis, splinter hemorrhages, oil stains, and nail pitting were evaluated at baseline and at 3 and 6 months. Results: A statistically significant improvement between baseline and 6 months was observed in all patients: the mean (SD) NAPSI went from 14.3 (6.3; 95% CI, 11.74-16.92) to 2.3 (1.21; 95% CI, 1.84-2.3) while the median went from 15 to 2.5 (P = 0.007). The percentage improvement at the end of treatment was 87.9%. No adverse effects were observed. Conclusion: Our study shows the therapeutic potential of tazarotene ointment in nail psoriasis (AU)

Nail psoriasis: treatment with tazarotene 0.1% hydrophilic ointment.

INTRODUCTION: Nail involvement is common in psoriasis and has a considerable impact on patient quality of life. Its clinical presentation depends on which part of the nail is affected: the bed or the matrix. Fifty percent of patients report associated pain. In this study, we analyzed the safety and effectiveness of tazarotene 0.1% in a hydrophilic ointment in the treatment of nail psoriasis. MATERIAL AND METHODS: We performed an open observational study of 6 patients diagnosed with nail psoriasis. The patients applied a compounded preparation of tazarotene 0.1% ointment under occlusion every night for 6 months in their homes. They were not receiving any other topical or systemic treatments. Nail psoriasis severity (assessed using the Nail Psoriasis Severity Index [NAPSI]), subungual hyperkeratosis, onycholysis, splinter hemorrhages, oil stains, and nail pitting were evaluated at baseline and at 3 and 6 months. RESULTS: A statistically significant improvement between baseline and 6 months was observed in all patients: the mean (SD) NAPSI went from 14.3 (6.3; 95% CI, 11.74-16.92) to 2.3 (1.21; 95% CI, 1.84-2.3) while the median went from 15 to 2.5 (P = .007). The percentage improvement at the end of treatment was 87.9%. No adverse effects were observed. CONCLUSION: Our study shows the therapeutic potential of tazarotene ointment in nail psoriasis.