Gluteal Hidradenitis Suppurativa: Analysis of 83 Patients / Hidradenitis supurativa con afectación glútea: análisis de 83 pacientes

Background In 2013, Canoui-Poitrine et al. identified three hidradenitis suppurativa (HS) phenotypes by a latent class (LC) analysis, based on anatomical sites of involvement. Objective To improve the classification of the gluteal phenotype (LC3) patients given their diverse lesion types and differences in clinical profile. Material and methods We designed a bicentric study gathering all LC3 patients (n=83) from two hospitals. We conducted a two-step cluster analysis among them and also compared their characteristics with the rest of the HS patients (n=661). Results Compared with global HS series, LC3 patients were more frequently non-obese men, with smoking habit, an associated arthropathy, and a more frequent history of pilonidal sinus. The analysis of LC3 patients yielded two clusters: cluster 1 (38.3%) included elderly female patients, with later diagnosis of the disease and more sinus tracts; cluster 2 (61.7%) encompassed more men with earlier disease onset and more nodules and folliculitis lesions. Limitations The study's limitations include its retrospective nature, bicentric design, and small sample size. Conclusion The heterogeneous clinical presentation of HS makes it essential to have a good classification of the patients. Gluteal phenotype could actually be classified into two “subphenotypes” with a different clinical profiles and management (AU)

Antecedentes En 2013 Canoui-Poitrine et al. identificaron tres fenotipos de hidradenitis supurativa (HS) mediante un análisis de clases latentes (LC) basado en las regiones anatómicas afectadas. Objetivo Mejorar la clasificación de los pacientes con fenotipo glúteo (LC3) dados los diversos tipos de lesiones y diferencias en el perfil clínico. Material y métodos Diseñamos un estudio bicéntrico que incluyó a todos los pacientes con LC3 (n=83) de dos hospitales terciarios españoles. Realizamos un análisis de conglomerados en dos etapas dentro del grupo LC3 y también comparamos sus características con el resto de los pacientes con HS (n=661). Resultados En comparación con la serie global de HS, los pacientes con LC3 fueron más frecuentemente hombres no obesos, fumadores, con una artritis asociada y con mayor prevalencia de sinus pilonidal. El análisis de los pacientes LC3 resultó en dos grupos: el grupo 1 (38,3%), que incluía pacientes mayores, de sexo femenino, con diagnóstico más tardío de la enfermedad y más trayectos sinusales; y el grupo 2 (61,7%), que englobaba a más hombres con inicio temprano de la enfermedad y más nódulos y lesiones de foliculitis. Limitaciones Las limitaciones del estudio incluyen su naturaleza retrospectiva, el diseño bicéntrico y el tamaño muestral reducido. Conclusión La presentación clínica heterogénea de la HS hace que sea esencial disponer de una buena clasificación clínica de los pacientes. Como hemos visto, parece que el fenotipo glúteo podría clasificarse en dos «subfenotipos» con perfiles clínicos y, consecuentemente, enfoques terapéuticos diferentes (AU)

[Artículo traducido] Hidradenitis supurativa con afectación glútea: análisis de 83 pacientes / Gluteal Hidradenitis Suppurativa: Analysis of 83 Patients

Antecedentes En 2013 Canoui-Poitrine et al. identificaron tres fenotipos de hidradenitis supurativa (HS) mediante un análisis de clases latentes (LC) basado en las regiones anatómicas afectadas. Objetivo Mejorar la clasificación de los pacientes con fenotipo glúteo (LC3) dados los diversos tipos de lesiones y diferencias en el perfil clínico. Material y métodos Diseñamos un estudio bicéntrico que incluyó a todos los pacientes con LC3 (n=83) de dos hospitales terciarios españoles. Realizamos un análisis de conglomerados en dos etapas dentro del grupo LC3 y también comparamos sus características con el resto de los pacientes con HS (n=661). Resultados En comparación con la serie global de HS, los pacientes con LC3 fueron más frecuentemente hombres no obesos, fumadores, con una artritis asociada y con mayor prevalencia de sinus pilonidal. El análisis de los pacientes LC3 resultó en dos grupos: el grupo 1 (38,3%), que incluía pacientes mayores, de sexo femenino, con diagnóstico más tardío de la enfermedad y más trayectos sinusales; y el grupo 2 (61,7%), que englobaba a más hombres con inicio temprano de la enfermedad y más nódulos y lesiones de foliculitis. Limitaciones Las limitaciones del estudio incluyen su naturaleza retrospectiva, el diseño bicéntrico y el tamaño muestral reducido. Conclusión La presentación clínica heterogénea de la HS hace que sea esencial disponer de una buena clasificación clínica de los pacientes. Como hemos visto, parece que el fenotipo glúteo podría clasificarse en dos «subfenotipos» con perfiles clínicos y, consecuentemente, enfoques terapéuticos diferentes (AU)

Background In 2013, Canoui-Poitrine et al. identified three hidradenitis suppurativa (HS) phenotypes by a latent class (LC) analysis, based on anatomical sites of involvement. Objective To improve the classification of the gluteal phenotype (LC3) patients given their diverse lesion types and differences in clinical profile. Material and methods We designed a bicentric study gathering all LC3 patients (n=83) from two hospitals. We conducted a two-step cluster analysis among them and also compared their characteristics with the rest of the HS patients (n=661). Results Compared with global HS series, LC3 patients were more frequently non-obese men, with smoking habit, an associated arthropathy, and a more frequent history of pilonidal sinus. The analysis of LC3 patients yielded two clusters: cluster 1 (38.3%) included elderly female patients, with later diagnosis of the disease and more sinus tracts; cluster 2 (61.7%) encompassed more men with earlier disease onset and more nodules and folliculitis lesions. Limitations The study's limitations include its retrospective nature, bicentric design, and small sample size. Conclusion The heterogeneous clinical presentation of HS makes it essential to have a good classification of the patients. Gluteal phenotype could actually be classified into two “subphenotypes” with a different clinical profiles and management (AU)

Efectos adversos cutáneos inmunomediados por los anti-TNF: revisión de 30 casos / Immune-Mediated Skin Reactions to Tumor Necrosis α Inhibitors: A Review of 30 Cases

Introducción Los fármacos biológicos inhibidores del factor de necrosis tumoral (TNF) alfa son usados para tratar diferentes enfermedades inflamatorias. A pesar de su adecuado perfil de seguridad, se han descrito reacciones paradójicas asociadas a estos tratamientos. Material y método Se ha realizado una revisión retrospectiva de los pacientes en tratamiento con un anti-TNF que hubiesen presentado una reacción paradójica con afectación cutánea visitados en el Servicio de Dermatología del Hospital Universitari Parc Taulí de Sabadell. Resultados Registramos 30 pacientes en tratamiento con un anti-TNF que desarrollaron un efecto adverso cutáneo inmunomediado en forma de psoriasis (90%), alopecia (6,7%) o dermatitis neutrofílica (3,3%). Adalimumab fue el fármaco más implicado (56,7%), seguido de infliximab (40%). La morfología de la reacción psoriasiforme más descrita es la generalizada en placas (62,9%), seguida de la pustulosis palmo-plantar (37%). El 43,3% de los pacientes mantuvieron el anti-TNF, y de ellos el 92,3% obtuvieron una resolución total y parcial. De los 5 pacientes que iniciaron otro anti-TNF, ninguno obtuvo una resolución total. De los 8 pacientes que cambiaron a un tratamiento biológico diferente al anti-TNF, el 62,5% obtuvieron una resolución total o parcial. Discusión La aparición de una reacción paradójica no siempre obliga al cambio de tratamiento biológico, puesto que se ha observado la resolución de las lesiones cutáneas con un tratamiento tópico y/o sistémico adicional en más de la mitad de los pacientes, sin necesidad de suspender el anti-TNF. Si la afectación es grave, se debe plantear el cambio de tratamiento biológico, siendo más eficaz iniciar un fármaco dirigido a una diana terapéutica distinta al anti-TNF (AU)

Background Tumor necrosis factor α (TNF) inhibitors are used to treat different inflammatory diseases. Although these biologics have an adequate safety profile, they have been associated with paradoxical reactions. Material and methods Retrospective review of patients on TNF inhibitor therapy who developed a paradoxical skin reaction and were seen at the dermatology department of Hospital Universitari Parc Taulí in Sabadell, Spain. Results We collected data on 30 patients under treatment with a TNF inhibitor who developed an immune-mediated skin reaction in the form of psoriasis (90%), alopecia (6.7%), or neutrophilic dermatitis (3.3%). The most common drugs involved were adalimumab (56.7%) and infliximab (40%). Psoriasiform reactions mostly manifested as generalized plaques (62.9%) or palmoplantar pustulosis (37%). Thirteen patients (43.3%) continued on the same TNF inhibitor and 12 of them (92.3%) achieved partial or complete resolution of lesions. Five patients were switched to a different TNF inhibitor, but none of them achieved complete resolution. Eight patients were switched to a biologic with a different target, and 5 of them (62.5%) achieved partial or complete resolution. Conclusions Paradoxical reactions during TNF inhibitor therapy do not always require a change of treatment. In our series, the addition of a topical and/or systemic treatment resolved the skin lesions in more than half of the patients, and switching to a drug with a different target was more effective. A change of strategy should be contemplated in more serious cases (AU)

[Translated article] Immune-Mediated Skin Reactions to Tumor Necrosis α Inhibitors: A Review of 30 Cases / Efectos adversos cutáneos inmunomediados por los anti-TNF: revisión de 30 casos

Background Tumor necrosis factor α (TNF) inhibitors are used to treat different inflammatory diseases. Although these biologics have an adequate safety profile, they have been associated with paradoxical reactions. Material and methods Retrospective review of patients on TNF inhibitor therapy who developed a paradoxical skin reaction and were seen at the dermatology department of Hospital Universitari Parc Taulí in Sabadell, Spain. Results We collected data on 30 patients under treatment with a TNF inhibitor who developed an immune-mediated skin reaction in the form of psoriasis (90%), alopecia (6.7%), or neutrophilic dermatitis (3.3%). The most common drugs involved were adalimumab (56.7%) and infliximab (40%). Psoriasiform reactions mostly manifested as generalized plaques (62.9%) or palmoplantar pustulosis (37%). Thirteen patients (43.3%) continued on the same TNF inhibitor and 12 of them (92.3%) achieved partial or complete resolution of lesions. Five patients were switched to a different TNF inhibitor, but none of them achieved complete resolution. Eight patients were switched to a biologic with a different target, and 5 of them (62.5%) achieved partial or complete resolution. Conclusions Paradoxical reactions during TNF inhibitor therapy do not always require a change of treatment. In our series, the addition of a topical and/or systemic treatment resolved the skin lesions in more than half of the patients, and switching to a drug with a different target was more effective. A change of strategy should be contemplated in more serious cases (AU)

Introducción Los fármacos biológicos inhibidores del factor de necrosis tumoral (TNF) alfa son usados para tratar diferentes enfermedades inflamatorias. A pesar de su adecuado perfil de seguridad, se han descrito reacciones paradójicas asociadas a estos tratamientos. Material y método Se ha realizado una revisión retrospectiva de los pacientes en tratamiento con un anti-TNF que hubiesen presentado una reacción paradójica con afectación cutánea visitados en el Servicio de Dermatología del Hospital Universitari Parc Taulí de Sabadell. Resultados Registramos 30 pacientes en tratamiento con un anti-TNF que desarrollaron un efecto adverso cutáneo inmunomediado en forma de psoriasis (90%), alopecia (6,7%) o dermatitis neutrofílica (3,3%). Adalimumab fue el fármaco más implicado (56,7%), seguido de infliximab (40%). La morfología de la reacción psoriasiforme más descrita es la generalizada en placas (62,9%), seguida de la pustulosis palmo-plantar (37%). El 43,3% de los pacientes mantuvieron el anti-TNF, y de ellos el 92,3% obtuvieron una resolución total y parcial. De los 5 pacientes que iniciaron otro anti-TNF, ninguno obtuvo una resolución total. De los 8 pacientes que cambiaron a un tratamiento biológico diferente al anti-TNF, el 62,5% obtuvieron una resolución total o parcial. Discusión La aparición de una reacción paradójica no siempre obliga al cambio de tratamiento biológico, puesto que se ha observado la resolución de las lesiones cutáneas con un tratamiento tópico y/o sistémico adicional en más de la mitad de los pacientes, sin necesidad de suspender el anti-TNF. Si la afectación es grave, se debe plantear el cambio de tratamiento biológico, siendo más eficaz iniciar un fármaco dirigido a una diana terapéutica distinta al anti-TNF (AU)

Gluteal Hidradenitis Suppurativa: Analysis of 83 Patients. / [Artículo traducido] Hidradenitis supurativa con afectación glútea: análisis de 83 pacientes.

BACKGROUND: In 2013, Canoui-Poitrine et al. identified three hidradenitis suppurativa (HS) phenotypes by a latent class (LC) analysis, based on anatomical sites of involvement. OBJECTIVE: To improve the classification of the gluteal phenotype (LC3) patients given their diverse lesion types and differences in clinical profile. MATERIAL AND METHODS: We designed a bicentric study gathering all LC3 patients (n=83) from two hospitals. We conducted a two-step cluster analysis among them and also compared their characteristics with the rest of the HS patients (n=661). RESULTS: Compared with global HS series, LC3 patients were more frequently non-obese men, with smoking habit, an associated arthropathy, and a more frequent history of pilonidal sinus. The analysis of LC3 patients yielded two clusters: cluster 1 (38.3%) included elderly female patients, with later diagnosis of the disease and more sinus tracts; cluster 2 (61.7%) encompassed more men with earlier disease onset and more nodules and folliculitis lesions. LIMITATIONS: The study's limitations include its retrospective nature, bicentric design, and small sample size. CONCLUSION: The heterogeneous clinical presentation of HS makes it essential to have a good classification of the patients. Gluteal phenotype could actually be classified into two "subphenotypes" with a different clinical profiles and management.

[Translated article] Immune-Mediated Skin Reactions to Tumor Necrosis α Inhibitors: A Review of 30 Cases.

BACKGROUND: Tumor necrosis factor α (TNF) inhibitors are used to treat different inflammatory diseases. Although these biologics have an adequate safety profile, they have been associated with paradoxical reactions. MATERIAL AND METHODS: Retrospective review of patients on TNF inhibitor therapy who developed a paradoxical skin reaction and were seen at the dermatology department of Hospital Universitari Parc Taulí in Sabadell, Spain. RESULTS: We collected data on 30 patients under treatment with a TNF inhibitor who developed an immune-mediated skin reaction in the form of psoriasis (90%), alopecia (6.7%), or neutrophilic dermatitis (3.3%). The most common drugs involved were adalimumab (56.7%) and infliximab (40%). Psoriasiform reactions mostly manifested as generalized plaques (62.9%) or palmoplantar pustulosis (37%). Thirteen patients (43.3%) continued on the same TNF inhibitor and 12 of them (92.3%) achieved partial or complete resolution of lesions. Five patients were switched to a different TNF inhibitor, but none of them achieved complete resolution. Eight patients were switched to a biologic with a different target, and 5 of them (62.5%) achieved partial or complete resolution. CONCLUSIONS: Paradoxical reactions during TNF inhibitor therapy do not always require a change of treatment. In our series, the addition of a topical and/or systemic treatment resolved the skin lesions in more than half of the patients, and switching to a drug with a different target was more effective. A change of strategy should be contemplated in more serious cases.

Immune-Mediated Skin Reactions to Tumor Necrosis α Inhibitors: A Review of 30 Cases. / Efectos adversos cutáneos inmunomediados por los anti-TNF: revisión de 30 casos.

BACKGROUND: Tumor necrosis factor α (TNF) inhibitors are used to treat different inflammatory diseases. Although these biologics have an adequate safety profile, they have been associated with paradoxical reactions. MATERIAL AND METHODS: Retrospective review of patients on TNF inhibitor therapy who developed a paradoxical skin reaction and were seen at the dermatology department of Hospital Universitari Parc Taulí in Sabadell, Spain. RESULTS: We collected data on 30 patients under treatment with a TNF inhibitor who developed an immune-mediated skin reaction in the form of psoriasis (90%), alopecia (6.7%), or neutrophilic dermatitis (3.3%). The most common drugs involved were adalimumab (56.7%) and infliximab (40%). Psoriasiform reactions mostly manifested as generalized plaques (62.9%) or palmoplantar pustulosis (37%). Thirteen patients (43.3%) continued on the same TNF inhibitor and 12 of them (92.3%) achieved partial or complete resolution of lesions. Five patients were switched to a different TNF inhibitor, but none of them achieved complete resolution. Eight patients were switched to a biologic with a different target, and 5 of them (62.5%) achieved partial or complete resolution. CONCLUSIONS: Paradoxical reactions during TNF inhibitor therapy do not always require a change of treatment. In our series, the addition of a topical and/or systemic treatment resolved the skin lesions in more than half of the patients, and switching to a drug with a different target was more effective. A change of strategy should be contemplated in more serious cases.

Gluteal Hidradenitis Suppurativa: Analysis of 83 Patients.

BACKGROUND: In 2013, Canoui-Poitrine et al. identified three hidradenitis suppurativa (HS) phenotypes by a latent class (LC) analysis, based on anatomical sites of involvement. OBJECTIVE: To improve the classification of the gluteal phenotype (LC3) patients given their diverse lesion types and differences in clinical profile. MATERIAL AND METHODS: We designed a bicentric study gathering all LC3 patients (n=83) from two hospitals. We conducted a two-step cluster analysis among them and also compared their characteristics with the rest of the HS patients (n=661). RESULTS: Compared with global HS series, LC3 patients were more frequently non-obese men, with smoking habit, an associated arthropathy, and a more frequent history of pilonidal sinus. The analysis of LC3 patients yielded two clusters: cluster 1 (38.3%) included elderly female patients, with later diagnosis of the disease and more sinus tracts; cluster 2 (61.7%) encompassed more men with earlier disease onset and more nodules and folliculitis lesions. LIMITATIONS: The study's limitations include its retrospective nature, bicentric design, and small sample size. CONCLUSION: The heterogeneous clinical presentation of HS makes it essential to have a good classification of the patients. Gluteal phenotype could actually be classified into two "subphenotypes" with a different clinical profiles and management.

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.

Lesiones pernióticas y acrales en España durante el confinamiento por COVID: análisis retrospectivo de 12 casos / Chilblain and Acral Purpuric Lesions in Spain during Covid Confinement: Retrospective Analysis of 12 Cases

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