The Effectiveness of Guselkumab in Patients With Hidradenitis Suppurativa Under Clinical Practice Conditions: A Spanish Multicentre Retrospective Study / fectividad de guselkumab en pacientes con hidradenitis supurativa en condiciones de práctica clínica: estudio retrospectivo y multicéntrico en España

Background Hidradenitis suppurativa (HS) is a chronic skin condition causing lesions in which high levels of interleukin (IL)-23 and T-helper 17 cells are found. Adalimumab remains the only approved treatment. Guselkumab, an antibody targeting the p19 protein subunit of extracellular IL-23, is approved for the treatment of moderate–severe psoriasis, but evidence on its efficacy in treating HS is limited.Objectives To assess the effectiveness and safety of guselkumab in treating moderate–severe HS under clinical practice conditions. Methods A multicentre retrospective observational study was carried out in 13 Spanish Hospitals including adult HS patients treated with guselkumab within a compassionate use programme (March 2020–March 2022). Data referred to patient demographic and clinical characteristics at treatment initiation (baseline), patient-reported outcomes (Numerical Pain Rating Scale [NPRS] and Dermatology Life Quality Index [DLQI]), physician scores (International Hidradenitis Suppurativa Severity Score System [IHS4], HS Physical Global Score [HS-PGA] and Hidradenitis Suppurativa Clinical Response [HiSCR]) were recorded at baseline and at 16, 24, and 48 weeks of treatment. Results A total of 69 patients were included. Most (84.10%) had severe HS (Hurley III) and had been diagnosed for over ten years (58.80%). The patients had been subjected to multiple non-biological (mean 3.56) or biological (mean 1.78) therapies, and almost 90% of those treated with biologics had received adalimumab (AU)

Antecedentes La hidradenitis supurativa (HS) es una situación cutánea crónica que causa lesiones en las que se encuentran altos niveles de interleucina (IL)-23 y células TH-17 colaboradoras, siendo adalimumab el único tratamiento aprobado. Guselkumab, un anticuerpo que focaliza la subunidad de la proteína p19 de IL-23 extracelular, ha sido aprobado para tratar la psoriasis de moderada a severa, siendo limitada la evidencia sobre su eficacia en el tratamiento de la HS. Objetivos Evaluar la efectividad y seguridad de guselkumab para el tratamiento de la HS de moderada a severa, en condiciones de práctica clínica. Métodos Se llevó a cabo un estudio observacional retrospectivo y multicéntrico en 13 hospitales españoles, que incluyó pacientes adultos de HS tratados con guselkumab, dentro de un programa de uso compasivo (de marzo de 2020 a marzo de 2022). Se registraron al inicio y a las 16, 24 y 48 semanas de tratamiento los datos referentes a las características demográficas y clínicas de los pacientes, los resultados reportados por el paciente (Numerical Pain Rating Scale [NPRS] y Dermatology Life Quality Index [DLQI]), puntuaciones del facultativo (International Hidradenitis Suppurativa Severity Score System [IHS4], HS Physical Global Score [HS-PGA] e Hidradenitis Suppurativa Clinical Response [HiSCR]). Resultados Se incluyó un total de 69 pacientes, de los cuales la mayoría (84,10%) tenían HS severa (Hurley III) y habían sido diagnosticados hacía más de 10 años (58,80%). Dichos pacientes habían sido sometidos a múltiples terapias no biológicas (media 3,56) o biológicas (media 1,78), y casi el 90% de los tratados con biológicos habían recibido adalimumab. Se observó una reducción significativa de las puntuaciones IHS4, HS-PGA, NPRS y DLQI desde el inicio hasta las 48 semanas del tratamiento con guselkumab (total p<0,01). Se logró HiSCR en el 58,33% y el 56,52% de los pacientes, a las 16 y 24 semanas, respectivamente (AU)

[Artículo traducido] Efectividad de guselkumab en pacientes con hidradenitis supurativa en condiciones de práctica clínica: estudio retrospectivo y multicéntrico en España / The Effectiveness of Guselkumab in Patients With Hidradenitis Suppurativa Under Clinical Practice Conditions: A Spanish Multicentre Retrospective Study

Antecedentes La hidradenitis supurativa (HS) es una situación cutánea crónica que causa lesiones en las que se encuentran altos niveles de interleucina (IL)-23 y células TH-17 colaboradoras, siendo adalimumab el único tratamiento aprobado. Guselkumab, un anticuerpo que focaliza la subunidad de la proteína p19 de IL-23 extracelular, ha sido aprobado para tratar la psoriasis de moderada a severa, siendo limitada la evidencia sobre su eficacia en el tratamiento de la HS. Objetivos Evaluar la efectividad y seguridad de guselkumab para el tratamiento de la HS de moderada a severa, en condiciones de práctica clínica. Métodos Se llevó a cabo un estudio observacional retrospectivo y multicéntrico en 13 hospitales españoles, que incluyó pacientes adultos de HS tratados con guselkumab, dentro de un programa de uso compasivo (de marzo de 2020 a marzo de 2022). Se registraron al inicio y a las 16, 24 y 48 semanas de tratamiento los datos referentes a las características demográficas y clínicas de los pacientes, los resultados reportados por el paciente (Numerical Pain Rating Scale [NPRS] y Dermatology Life Quality Index [DLQI]), puntuaciones del facultativo (International Hidradenitis Suppurativa Severity Score System [IHS4], HS Physical Global Score [HS-PGA] e Hidradenitis Suppurativa Clinical Response [HiSCR]). Resultados Se incluyó un total de 69 pacientes, de los cuales la mayoría (84,10%) tenían HS severa (Hurley III) y habían sido diagnosticados hacía más de 10 años (58,80%). Dichos pacientes habían sido sometidos a múltiples terapias no biológicas (media 3,56) o biológicas (media 1,78), y casi el 90% de los tratados con biológicos habían recibido adalimumab. Se observó una reducción significativa de las puntuaciones IHS4, HS-PGA, NPRS y DLQI desde el inicio hasta las 48 semanas del tratamiento con guselkumab (total p<0,01). Se logró HiSCR en el 58,33% y el 56,52% de los pacientes, a las 16 y 24 semanas, respectivamente (AU)

Background Hidradenitis suppurativa (HS) is a chronic skin condition causing lesions in which high levels of interleukin (IL)-23 and T-helper 17 cells are found. Adalimumab remains the only approved treatment. Guselkumab, an antibody targeting the p19 protein subunit of extracellular IL-23, is approved for the treatment of moderate–severe psoriasis, but evidence on its efficacy in treating HS is limited.Objectives To assess the effectiveness and safety of guselkumab in treating moderate–severe HS under clinical practice conditions. Methods A multicentre retrospective observational study was carried out in 13 Spanish Hospitals including adult HS patients treated with guselkumab within a compassionate use programme (March 2020–March 2022). Data referred to patient demographic and clinical characteristics at treatment initiation (baseline), patient-reported outcomes (Numerical Pain Rating Scale [NPRS] and Dermatology Life Quality Index [DLQI]), physician scores (International Hidradenitis Suppurativa Severity Score System [IHS4], HS Physical Global Score [HS-PGA] and Hidradenitis Suppurativa Clinical Response [HiSCR]) were recorded at baseline and at 16, 24, and 48 weeks of treatment. Results A total of 69 patients were included. Most (84.10%) had severe HS (Hurley III) and had been diagnosed for over ten years (58.80%). The patients had been subjected to multiple non-biological (mean 3.56) or biological (mean 1.78) therapies, and almost 90% of those treated with biologics had received adalimumab (AU)

The Effectiveness of Guselkumab in Patients With Hidradenitis Suppurativa Under Clinical Practice Conditions: A Spanish Multicentre Retrospective Study. / [Artículo traducido] Efectividad de guselkumab en pacientes con hidradenitis supurativa en condiciones de práctica clínica: estudio retrospectivo y multicéntrico en España.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic skin condition causing lesions in which high levels of interleukin (IL)-23 and T-helper17 cells are found. Adalimumab remains the only approved treatment. Guselkumab, an antibody targeting the p19 protein subunit of extracellular IL-23, is approved for the treatment of moderate-severe psoriasis, but evidence on its efficacy in treating HS is limited. OBJECTIVES: To assess the effectiveness and safety of guselkumab in treating moderate-severe HS under clinical practice conditions. METHODS: A multicentre retrospective observational study was carried out in 13 Spanish hospitals including adult HS patients treated with guselkumab within a compassionate use programme (March 2020-March 2022). Data referred to patient demographic and clinical characteristics at treatment initiation (baseline), patient-reported outcomes (Numerical Pain Rating Scale [NPRS] and Dermatology Life Quality Index [DLQI]), physician scores (International Hidradenitis Suppurativa Severity Score System [IHS4], HS Physical Global Score [HS-PGA] and Hidradenitis Suppurativa Clinical Response [HiSCR]) were recorded at baseline and at 16, 24, and 48weeks of treatment. RESULTS: A total of 69 patients were included. Most (84.10%) had severe HS (HurleyIII) and had been diagnosed for over ten years (58.80%). The patients had been subjected to multiple non-biological (mean: 3.56) or biological (mean: 1.78) therapies, and almost 90% of those treated with biologics had received adalimumab. A significant decrease in IHS4, HS-PGA, NPRS, and DLQI scores was observed from baseline to 48weeks of guselkumab treatment (all P<.01). HiSCR was achieved in 58.33% and 56.52% of the patients at 16 and 24weeks, respectively. Overall, 16 patients discontinued treatment, mostly due to inefficacy (n=7) or loss of efficacy (n=3). No serious adverse events were observed. CONCLUSIONS: Our results indicate that guselkumab may be a safe and effective therapeutic alternative for patients with severe HS that fail to respond to other biologics.

The Effectiveness of Guselkumab in Patients With Hidradenitis Suppurativa Under Clinical Practice Conditions: A Spanish Multicentre Retrospective Study.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic skin condition causing lesions in which high levels of interleukin (IL)-23 and T-helper 17 cells are found. Adalimumab remains the only approved treatment. Guselkumab, an antibody targeting the p19 protein subunit of extracellular IL-23, is approved for the treatment of moderate-severe psoriasis, but evidence on its efficacy in treating HS is limited. OBJECTIVES: To assess the effectiveness and safety of guselkumab in treating moderate-severe HS under clinical practice conditions. METHODS: A multicentre retrospective observational study was carried out in 13 Spanish Hospitals including adult HS patients treated with guselkumab within a compassionate use programme (March 2020-March 2022). Data referred to patient demographic and clinical characteristics at treatment initiation (baseline), patient-reported outcomes (Numerical Pain Rating Scale [NPRS] and Dermatology Life Quality Index [DLQI]), physician scores (International Hidradenitis Suppurativa Severity Score System [IHS4], HS Physical Global Score [HS-PGA] and Hidradenitis Suppurativa Clinical Response [HiSCR]) were recorded at baseline and at 16, 24, and 48 weeks of treatment. RESULTS: A total of 69 patients were included. Most (84.10%) had severe HS (Hurley III) and had been diagnosed for over ten years (58.80%). The patients had been subjected to multiple non-biological (mean 3.56) or biological (mean 1.78) therapies, and almost 90% of those treated with biologics had received adalimumab. A significant decrease in IHS4, HS-PGA, NPRS, and DLQI scores was observed from baseline to 48 weeks of guselkumab treatment (all p<0.01). HiSCR was achieved in 58.33% and 56.52% of the patients at 16 and 24 weeks, respectively. Overall, 16 patients discontinued treatment, mostly due to inefficacy (n=7) or loss of efficacy (n=3). No serious adverse events were observed. CONCLUSIONS: Our results indicate that guselkumab may be a safe and effective therapeutic alternative for patients with severe HS that fail to respond to other biologics.

Pitiriasis «rubra pilaris» localizada / Pityriasis rubra pilaris localized

Incluimos la pitiriasis rubra pilaris (PRP) dentro del grupo de las dermatosis papuloescamosas hiperqueratósicas de origen desconocido. Aunque poco frecuente, se han constatado dos picos de incidencia en la primera y en la quinta década de la vida, lo cual determina su clasificación en cinco grandes grupos, los dos primeros típicos en la edad adulta y los tres restantes en la edad juvenil. En los últimos años se ha incluido un sexto grupo, asociado al virus de la inmunodeficiencia humana (VIH), independientemente de la edad. No existe un tratamiento estandarizado para la PRP, pero por lo general en casos localizados suelen emplearse tratamientos tópicos, entre los que incluimos los corticoides, los derivados de la vitamina D o los retinoides; en casos más extensos podría recurrirse a tratamientos sistémicos, y como primera elección los retinoides orales(AU)

Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous keratotic dermatosis of unknown origin. It has a bimodal distribution of age onset concentrating in the first and fifth decades. Classification of PRP includes 5 types: types I and II represent the forms of adult PRP, respectively, whereas types III, IV, and V are seen in juveniles. In recent years, a type VI PRP associated with HIV infection has been proposed. A universal standard treatment for PRP is lacking. Topical therapy is the treatment of choice for patients with mild type III and type IV PRP, including corticoids, vitamin D analogue ortopical retinoids. Systemic therapy is reserved for patients with severe type III and type V. Acitretin and isotretinoin are the treatment of choice(AU)

Síndrome de Peutz-Jeghers / Peutz-Jeghers syndrome

Presentamos el caso de un niño de 6 años de edad con lesioneshiperpigmentadas en la región perioral. El padre del pacientehabía sido diagnosticado de síndrome de Peutz-Jeghers (SPJ)mediante un estudio genético. Las lesiones cutáneas, junto conlos antecedentes familiares, fueron la clave para el diagnósticotemprano de la enfermedad. El SPJ es una entidad rara, caracterizadapor la aparición de lentigos periorificiales y póliposgastrointestinales. Histológicamente, estos pólipos son hamartomasque pueden llegar a malignizarse. Además, el SPJ seasocia al desarrollo de tumores extraintestinales (mama, endometrio,ovario, testículo, páncreas...). Por ello, es necesariorealizar un diagnóstico precoz y un control periódico de laspersonas que padecen este síndrome y sus familiares(AU)

We report the case of a 6-year-old child with hyperpigmentedlesions in perioral region. His father had been diagnosed ofPeutz-Jeghers syndrome (PJS) by genetic testing. PJS is a rareentity characterized by the presence of hyperpigmented periorificiallesions and gastrointestinal polyps. Histologically, thesepolyps are hamartomas that can become malignant. Moreover,PJS is associated with the development of nongastrointestinalcancer (breast, endometrium, ovary, testicle, pancreas...). It istherefore necessary to make an early diagnosis and periodicmonitoring of the patients with this syndrome and their families(AU)

Actitud expectante como tratamiento eficaz en un caso de múltiples lesiones por «Molluscum contagiosum» / Attitude expectant as effective treatment in Molluscum contagiosum

El molusco contagioso es una infección cutánea frecuente causada por un virus de la familia de los poxvirus, que afecta principalmente a los niños. La enfermedad puede transmitirse por contacto directo a través de la piel, por fómites contaminados o por autoinoculación. La infección se resuelve habitualmente de forma espontánea en pacientes inmunocompetentes, en un tiempo que puede oscilar entre meses y años. Existe un debate continuo sobre si se debe tratar activamente o mantener una actitud expectante(AU)

Molluscum contagiosum is a common skin infection, caused by a poxvirus, that affect mainly children. The disease can be transmitted by direct contact, fomites, or auto-inoculation. The infection will usually resolve within months or years in people with normal immunity. There has been a continous discussion about whether physicians should treat Molluscum contagiosum actively or not(AU)

Telangiectasia nevoide unilateral / Unilateral nevoid telangiectasia

La telangiectasia nevoide unilateral se define por la aparición progresiva de telangiectacias localizadas de manera blaschkoide o unilateral en un dermatoma cutáneo, habitualmente en la cabeza, el cuello y los miembros superiores. Las lesiones suelen comenzar en torno a la pubertad y son más habituales en pacientes de sexo femenino. Es una patología benigna con repercusiones meramente estéticas, pero en ocasiones puede asociarse a hepatopatía o a enfermedades autoinmunes, como el lupus eritematoso sistémico. El tratamiento consiste en láser colorante pulsado(AU)

Unilateral nevoid telangiectasia is a disorder characterized by a progressive occurrence of telangiectases on the skin. They are usually arranged in an unilateral or Blaschkoid pattern on the head, the neck or the upper limb. The disease appears around puberty and has a female predilection. Unilateral nevoid telangiectasia is a benign disorder but it has also been described in association with hepatopathy or with a connective tissue disease like lupus erythematosus. The pulsed dye laser is the treatment of choice(AU)

Cutis marmorata telangiectásica congénita / Cutis marmorata telangiectatica congenita

La cutis marmorata telangiectásica congénita es una malformación vascular de bajo flujo, caracterizada por su distintivo aspecto violáceo y reticulado grueso, como el mármol. Generalmente, está presente en el nacimiento, se hace más pronunciada en las primeras semanas de vida y suele perder intensidad de forma progresiva durante los 2 primeros años de vida. El diagnóstico se establece a partir de los hallazgos clínicos. Se han publicado numerosas anomalías asociadas con la cutis marmorata telangiectásica congénita, como la asimetría corporal y otras anomalías vasculares. Presentamos un nuevo caso de cutis marmorata telangiectásica congénita en un bebé de 2 meses de edad, que asociaba una hipoplasia de la extremidad inferior derecha y dos malformaciones capilares (AU)

Cutis marmorata telangiectatica congenita is a low-flow vascular malformation that is characterized by its distinctive violaceous coarsely reticulated marbled pattern. It is generally present at birth, may become more vivid within the first weeks, and typically slowly fades thereafter, particularly during the first years of life. The diagnosis is established on clinical grounds. Additional anomalies have been reported in association with cutis marmorata telangiectatica congenita, including body asymmetry and other vascular anomalies. We present a new case of cutis marmorata telangiectatica congenita in a 2 month-year-old baby, with an hypoplasia of the right limb and two capillary malformations associated (AU)

Exantema periflexural asimétrico / Asymmetric periflexural exanthem

El exantema periflexural asimétrico en la infancia es un exantema de causa desconocida, caracterizado por una erupción maculopapulosa escarlatiniforme, con un crecimiento centrífugo. Comienza típicamente cerca de la axila o la región inguinal, de forma predominantemente unilateral, aunque a medida quela erupción progresa suele extenderse de forma bilateral. Se resuelve de manera espontánea en 1-3 semanas. Presentamos el caso de un niño de 4 meses de edad con esta erupción (AU)

Asymmetric periflexural exanthem of childhood is an exanthema of unknown a etiology. It is described as a macular-papular periflexural scarlatiniform eruption with a centrifugal spreading. Tipically begins near an axilla or groin and retains a unilateral predominance, although it spreads to become bilateral as the eruption progresses. The eruption usually resolves in1-3 weeks without treatment. We report a 4-month-old boy with this exanthema (AU)

[Clinical outcomes in patients with psoriasis following discontinuation of efalizumab due to suspension of marketing authorization]. / Evolución clínica de los pacientes psoriásicos tratados con efalizumab al suspender el fármaco.

INTRODUCTION: In February 2009, the European Medicines Agency suspended the marketing authorization for efalizumab after 3 confirmed cases of progressive multifocal leukoencephalopathy were reported. To assess the consequences of this decision, we performed a prospective follow-up study of patients in our department who were being treated with efalizumab at the time and compared clinical outcomes with data from the literature. PATIENTS AND METHODS: Thirty-two patients (28 with plaque psoriasis and 4 with palmoplantar psoriasis) were enrolled between February and March 2009. We recorded psoriasis area and severity index (PASI) scores at the moment of efalizumab discontinuation, at 6 weeks post-discontinuation, and at 3-monthly intervals thereafter. PASI scores prior to treatment with efalizumab were also noted. For patients who experienced rebounds with generalized psoriasis, we noted the time that had elapsed since efalizumab discontinuation and the treatment they were receiving. RESULTS: Even though 92.8% of the patients were considered good responders (>75% reduction in PASI score), 25% of the group (8/32) experienced rebound and 15.7% (5/32) experienced relapse. The percentage of patients in whom rebound was observed on transition therapy was 18% (2/11) for cyclosporin, 50% (1/2) for methotrexate, 50% (1/2) for adalimumab, 50% (1/2) for etanercept, and 27% (3/11) for topical treatment. CONCLUSIONS: We observed a very high rate of rebound and generalized inflammation in patients whose disease had previously been well controlled for several years.

Evolución clínica de los pacientes psoriásicos tratados con efalizumab al suspender el fármaco / Clinical Outcomes in Patients With Psoriasis Following Discontinuation of Efalizumab due to Suspension of Marketing Authorization

Introducción: En febrero de 2009 la Agencia Europea de Evaluación de Medicamentos (EMEA) suspendió la comercialización de efalizumab por la notificación de tres casos confirmados de leucoencefalopatía multifocal progresiva (LMP). Nos planteamos realizar un estudio prospectivo de seguimiento de los pacientes tratados en nuestro Servicio con efalizumab en el momento de la suspensión y comparar la evolución con las perspectivas publicadas en la literatura. Pacientes y métodos: Se incluyeron 32 pacientes (28 con psoriasis en placas y 4 con psoriasis palmoplantar) entre febrero y marzo de 2009. Se recogió el Psoriasis Area and Severity Index (PASI) previo al comienzo del tratamiento con efalizumab, en el momento de la suspensión, a las 6 semanas y posteriormente cada tres meses. En el caso de los pacientes que presentaron brotes generalizados se recogió el tiempo transcurrido desde la suspensión y el manejo terapéutico que se realizó. Resultados: A pesar de que el 92,8% de los pacientes correspondían a buenos respondedores (mejoría PASI >75), presentaron rebote un 25% de los sujetos (8/32) y recaídas un 15,7% (5/32). Con respecto a la terapia de transición presentaron rebote un 18% de los pacientes (2/11) con ciclosporina, un 50% (1/2) con metotrexato, un 50% (1/2) con adalimumab, un 50% (1/2) con etanercept y un 27% (3/11) de los que recibieron tratamiento tópico. Conclusiones: Hemos encontrado un porcentaje muy alto de rebote y formas generalizadas inflamatorias en pacientes que habían conseguido un buen control de la psoriasis durante varios años (AU)

Introduction: In February 2009, the European Medicines Agency suspended the marketing authorization for efalizumab after 3 confirmed cases of progressive multifocal leukoencephalopathy were reported. To assess the consequences of this decision, we performed a prospective follow-up study of patients in our department who were being treated with efalizumab at the time and compared clinical outcomes with data from the literature. Patients and methods: Thirty-two patients (28 with plaque psoriasis and 4 with palmoplantar psoriasis) were enrolled between February and March 2009. We recorded psoriasis area and severity index (PASI) scores at the moment of efalizumab discontinuation, at 6 weeks post-discontinuation, and at 3-monthly intervals thereafter. PASI scores prior to treatment with efalizumab were also noted. For patients who experienced rebounds with generalized psoriasis, we noted the time that had elapsed since efalizumab discontinuation and the treatment they were receiving. Results: Even though 92.8% of the patients were considered good responders (>75% reduction in PASI score), 25% of the group (8/32) experienced rebound and 15.7% (5/32) experienced relapse. The percentage of patients in whom rebound was observed on transition therapy was 18% (2/11) for cyclosporin, 50% (1/2) for methotrexate, 50% (1/2) for adalimumab, 50% (1/2) for etanercept, and 27% (3/11) for topical treatment. Conclusions: We observed a very high rate of rebound and generalized inflammation in patients whose disease had previously been well controlled for several years (AU)

Enfermedad mano-pie-boca / Hand, foot and mouth disease

La enfermedad mano-pie-boca es una entidad nosológica de causa viral, que se caracteriza por la presencia de lesiones cutáneas en la mucosa oral y las regiones acrales. En la mayoría de las ocasiones el agente causal son los virus Coxsackie, miembros de la familia picornavirus. La infección se presenta habitualmente de forma esporádica, si bien pueden existir epidemias. El tratamiento es sintomático y el pronóstico es excelente, aunque se han descrito complicaciones neurológicas y casos mortales (AU)

Hand, foot and mouth disease is a nosological entity of viralorig in characterised by cutaneous lesions of the oral mucosa and acral regions. In most cases the causal agent is the Coxsackie virus, a member of the Picornavirus family. The infection usually occurs sporadically, even though epidemics may occur. Its treatment is symptomatic and the prognosis is excellent, although there have been reports of neurological complications and fatal cases (AU)

Hipomelanosis macular progresiva / Progressive macular hypomelanosis

La hipomelanosis macular progresiva es una entidad poco conocida y, sin embargo, relativamente frecuente. Cursa con la aparición de máculas hipocrómicas asintomáticas en el tronco de adolescentes y adultos jóvenes, sin inflamación previa. La etiología de esta enfermedad es desconocida, pero se postula la posible intervención de cepas de Propionibacterium acnes. No tiene tratamiento eficaz, pero hay casos que responden a la fototerapia y la antibioterapia tópica (AU)

Progressive macular hypomelanosis is a little-known and yet relatively common condition. It presents with the appearance of asymptomatic hypochromic macules on the trunk of adolescents and young adults, without prior inflammation. This disease if of unknown a etiology, but is thought to involve the possible intervention of strains of Propionibacterium acnes. There is no efficient treatment for this disease, although certain cases respond to phototherapy and topical antibiotherapy (AU)

[Erythrodermic lichen planus pemphigoides]. / Liquen plano penfigoide eritrodérmico.

Lichen planus pemphigoides is a clinico-histological subtype of lichen planus in which there are bullous lesions similar to those of bullous pemphigoid. Lichen planus is included among the rare causes of erythroderma. We present a case of erythrodermic lichen planus pemphigoides in a 49-year-old female patient who satisfactorily responded to treatment with cyclosporine. A detailed physical examination and immunofluorescence study are key to the diagnosis of lichen planus pemphigoides.

Liquen plano penfigoide eritrodérmico / Erythrodermic lichen planus pemphigoides

El liquen plano penfigoide es un subtipo clínico-histológico de liquen plano en el que aparecen lesiones ampollosas similares a las del penfigoide ampolloso. El liquen plano se incluye entre las causas excepcionales de eritrodermia. Presentamos un caso de liquen plano penfigoide eritrodérmico en una paciente de 49 años que respondió satisfactoriamente al tratamiento con ciclosporina. Una minuciosa exploración física y el estudio mediante inmunofluorescencia son claves en el diagnóstico del liquen plano penfigoide

Lichen planus pemphigoides is a clinico-histological subtype of lichen planus in which there are bullous lesions similar to those of bullous pemphigoid. Lichen planus is included among the rare causes of erythroderma. We present a case of erythrodermic lichen planus pemphigoides in a 49-year-old female patient who satisfactorily responded to treatment with cyclosporine. A detailed physical examination and immunofluorescence study are key to the diagnosis of lichen planus pemphigoides

Epidermolysis bullosa acquisita: diagnosis by fluorescence overlay antigen mapping and clinical response to high-dose intravenous immunoglobulin.

Epidermolysis bullosa acquisita (EBA) is a chronic, subepidermal blistering disease characterized by the presence of autoantibodies to type VII collagen, located below the lamina densa of the basement membrane zone (BMZ). There is a large clinical and histological overlap between EBA and other subepidermal autoimmune bullous diseases, therefore, complex immunological techniques are required to make an accurate diagnosis. Therapy of EBA is also a difficult issue. Most patients do not respond to several common immunosuppressive agents. We describe a patient who has shown a good response to high-dose intravenous immunoglobulin therapy.

Reducción de la ansiedad en la antesala del quirófano en pacientes pediátricos / Reduction of anxiety in the anteroom of operating theatre in paediatric patients

Los estudios indican que la ansiedad antes de la intervención quirúrgica en pacientes pediátricos influye negativamente en la recuperación posthospitalaria. Por lo tanto, numerosas investigaciones se dirigen a paliar la ansiedad mediante diversas técnicas psicológicas en este momento tan crítico del niño o niña que va a ser operado. En nuestro caso, hemos aprovechado un recurso propio del Hospital de Sant Joan de Déu: el equipo de voluntariado que se encuentra en la antesala del quirófano para acompañar a los niños. El objetivo de nuestro estudio ha sido entrenar a dichas voluntarias (que no tenían ninguna formación específica para su labor) y ver el efecto en la ansiedad de los niños. Los sujetos que formaron parte de la investigación fueron 140 niños y niñas de entre 0 y 18 años. Los resultados indican que el entrenamiento no sólo ha ejercido cambios significativos de ciertas conductas de las voluntarias, sino que también se han conseguido cambios en las conductas relacionadas con la ansiedad prequirúrgica de los niños. Así consideramos recomendable el acompañamiento de los niños por parte de voluntariado entrenado (AU)

[Reduction of anxiety in the anteroom of the operating theater in pediatric patients]. / Reducción de la ansiedad en la antesala del quirófano en pacientes pediátricos.

Research indicates that anxiety before surgical intervention in paediatric patients negatively affects on post hospital recovery. Numerous investigations are, therefore, conducted to alleviate anxiety through different psychological techniques at this critical moment that the child is undergoing in surgery. In our case, we have considered one of the resources of the Sant Joan de Déu Hospital: a voluntary team that works in the anteroom of the operating theatre to keep the children company. The aim of our research has been to train these volunteers (who previously did not have any specific training for their task) and to observe the effect on the children's anxiety. The subjects of our study were 140 boys and girls between the ages of 0 and 18 years old. Results indicate that training not only produces significative changes on volunteers' behaviour, but also has resulted in the children's presurgical anxiety behaviour. In this way, we recommend that all children be accompanied by a trained volunteer.