Treatment of Behçet disease with oral roflumilast, an observational study.

INTRODUCTION: Mucocutaneous Behcet's disease is often a therapeutic challenge. Roflumilast has shown promise in other inflammatory dermatological conditions. The objective of this study is to evaluate the characteristics, effectiveness and safety of roflumilast in the treatment of Behçet's Disease-associated aphthosis in routine clinical practice. METHODS: Single cohort ambispective observational study. 11 patients with Behçet disease treated with roflumilast participated. Data collection included demographic, clinical and outcome variables. Statistical analysis compared 12 weeks of treatment with roflumilast with a previous period without treatment and with a period with the previous treatment. RESULTS: During treatment with roflumilast, a reduction in flare-ups and oral ulcers was observed compared to the untreated period and the previous treatment period. A reduction in genital ulcers, pain and ulcer duration was observed between the Whitout treatment period and the Roflumilast treatment period.Adverse effects occurred in 54% of patients, most of which were self-limiting or manageable with dose adjustment. No patient withdrew treatment. DISCUSSION: Roflumilast appears a promising option in the treatment of Behçet's disease with favourable effectiveness, safety and tolerability profiles. Although further research is needed, roflumilast offers a promising treatment option for Behçet's Disease-associated aphthosis, which could improve patients' quality of life and address unmet therapeutic needs.

Fiebre botonosa mediterránea en niño de 3 años tratada con doxiciclina / Mediterranean spotted fever in a 3-year-old boy treated with doxycycline

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Allergic and photoallergic contact dermatitis to chlorpromazine.

Chlorpromazine is known to produce both systemic phototoxic and photoallergic reactions. However, it may also cause photoallergic contact dermatitis and, albeit exceptionally, allergic contact dermatitis (ACD). We present a series of photoallergic contact dermatitis and ACD to chlorpromazine diagnosed at a tertiary centre cutaneous allergy unit between 1980 and 2019.

Allergic contact dermatitis to chlorhexidine in pediatric patients.

Chlorhexidine is one of the most widely used antiseptics in our environment. Allergic contact dermatitis to chlorhexidine is common in adults, but is quite rare in the pediatric population. Its diagnosis is important because it can mimic other dermatoses common during childhood, and it can also lead to acute immediate hypersensitivity reactions following successive exposures.

European photopatch test baseline series: A 3-year experience.

BACKGROUND: In 2012, a consensus was reached regarding a baseline photopatch test series on the basis of the results of a European multicentre study. OBJECTIVES: To describe experience with the European photopatch test series. METHODS: A retrospective analysis of 116 patients tested with the European photopatch test series between 2014 and 2016 was performed. RESULTS: Fifty-five positive photopatch test reactions in 25 subjects were recorded, most commonly caused by the topical non-steroidal anti-inflammatory drugs ketoprofen, dexketoprofen, and etofenomate. Organic ultraviolet (UV) absorbers constituted the second main category of agents eliciting positive photopatch test reactions. Among UV absorbers, benozophenone-3 and octocrylene were the most frequent photoallergens. UV absorbers that have been introduced more recently rarely elicited positive photopatch test reactions. Positive patch test reactions were less commonly observed than positive photopatch test reactions, namely, 21 reactions in 14 patients. CONCLUSIONS: We present the largest clinical experience with the European photopatch test baseline series hitherto reported. The results are similar to those underlying the above consensus process, reaffirming the usefulness of this series.

Efficacy of Propranolol Between 6 and 12 Months of Age in High-Risk Infantile Hemangioma.

BACKGROUND AND OBJECTIVES: There is no consensus on optimal treatment duration for propranolol in infantile hemangioma (IH). We evaluated the efficacy and safety of oral propranolol solution administered for a minimum of 6 months up to a maximum of 12 months of age in high-risk IH. METHODS: This single-arm, open-label, phase 3 study was conducted in patients aged 35 to 150 days with high-risk IH in 10 hospitals between 2015 and 2017. The study comprised a 6-month initial treatment period (ITP) plus continuation up to 12 months of age if complete success was not achieved, a follow-up, and a retreatment period. Patients received oral propranolol twice daily (3 mg/kg per day). The primary end point was the success rate at the end of the ITP. Furthermore, the persistence of IH response and efficacy of retreatment was evaluated. RESULTS: The success rate after 6 months of treatment was 47%, increasing to 76% at the end of the ITP. Of the patients who achieved success, 68% sustained success for 3 months without treatment, and 24% required retreatment. Of the 8 patients who were retreated, 7 achieved success. Adverse events, reported by 80% of patients, were mild, which were expected in this population or known propranolol side effects. CONCLUSIONS: Oral propranolol administered beyond 6 months and up to 12 months of age meaningfully increases the success rate in high-risk IH. Success was sustained in most patients up to 3 months after stopping treatment. Retreatment was efficacious, and the safety profile satisfactory.

Eccema de manos en niños. Estudio clínico-epidemiológico de la población remitida a un hospital terciario / Hand eczema in children. Clinical and epidemiological study of the population referred to a tertiary hospital

INTRODUCCIÓN: El eccema de manos es una forma frecuente de eccema en adultos. Su diagnóstico en ocasiones es complejo debido a la existencia de diferentes clasificaciones diagnósticas. Existen pocos trabajos que estudien el eccema de manos y su clasificación en niños. MATERIAL Y MÉTODO: Se ha identificado a 389 niños entre 0 y 16 años remitidos a la Unidad de Alergia Cutánea de nuestro servicio para estudio con pruebas epicutáneas en el periodo 1996-2016. De entre todos los casos se han seleccionado 42 casos con dermatitis localizada exclusivamente en la mano (10,8% de todos los niños remitidos). En todos los casos se realizaron pruebas epicutáneas parchando la batería estándar, así como baterías adicionales en función de la sospecha clínica. Se recogieron datos epidemiológicos (edad, sexo, antecedentes de dermatitis atópica...), así como clínicos (localización de las lesiones). RESULTADOS: De los 42 niños remitidos con dermatitis de la mano, 25 (60,5%) eran niñas y 17 (40,5%) niños. La edad media de los pacientes con dermatitis de la mano fue de 10,6 ± 3,9 años. El diagnóstico definitivo tras la realización de pruebas epicutáneas fue dermatitis atópica en 15 casos, dermatitis alérgica de contacto en 14 pacientes, eccema endógeno vesiculoso en 6 casos, eccema endógeno hiperqueratósico en 5 casos y dermatitis irritativa de contacto en 2 casos. Los alérgenos detectados más frecuentes fueron tiomersal (9 casos), niquel (5 casos), mercurio (5 casos) y cobalto (4 casos). CONCLUSIÓN: El eccema de manos es una entidad frecuente en niños. La causa más frecuente es la dermatitis atópica, aunque no son infrecuentes los casos de dermatitis alérgica de contacto que se manifiestan como eccema de manos. Todo niño con eccema de manos en el que se sospeche una causa alérgica debe ser remitido para realización de pruebas epicutáneas

INTRODUCTION: Hand eczema is a frequent disease in adults. Diagnosing the cause of hand eczema is difficult due to different classifications. There is lack of evidence on hand eczema and its causes in children. MATERIAL AND METHOD: A total of 389 children between 0 and 16 years were identified between 1996 and 2016, from whom 42 (10.8%) with exclusively hand eczema were selected. In all cases a standard battery of epicutaneous patch tests was performed, as well as additional batteries depending on the clinical suspicion. The clinical and epidemiological features of these children were recorded and compared against children with eczema in other locations. RESULTS: The 42 children with hand eczema included 25 (60.5%) girls, and 17 (40.5%) boys, with a mean age of 10.6 +- 3.9 years, and did not differ from that of children with eczema in other locations. The definitive diagnosis after patch-testing was Atopic Dermatitis in 15 cases, Allergic Contact Dermatitis in 14 patients, Endogenous Vesiculous Eczema in 6 cases, Endogenous Hyperkeratotic Eczema in 5 cases, and Irritant Contact Dermatitis in 2 cases. The most frequent allergens detected were thiomersal (9 cases), nickel (5 cases), mercury (5 cases), and cobalt (4 cases). CONCLUSION: Hand eczema is a common condition in children. The most common cause is atopic dermatitis, although cases of allergic contact dermatitis manifesting as hand eczema are not uncommon. Any child with eczema of hands in whom an allergic cause is suspected should be referred for patch- testing

Rothmund-Thomson Syndrome: Insights from New Patients on the Genetic Variability Underpinning Clinical Presentation and Cancer Outcome.

Biallelic mutations in RECQL4 gene, a caretaker of the genome, cause Rothmund-Thomson type-II syndrome (RTS-II) and confer increased cancer risk if they damage the helicase domain. We describe five families exemplifying clinical and allelic heterogeneity of RTS-II, and report the effect of pathogenic RECQL4 variants by in silico predictions and transcripts analyses. Complete phenotype of patients #39 and #42 whose affected siblings developed osteosarcoma correlates with their c.[1048_1049del], c.[1878+32_1878+55del] and c.[1568G>C;1573delT], c.[3021_3022del] variants which damage the helicase domain. Literature survey highlights enrichment of these variants affecting the helicase domain in patients with cancer outcome raising the issue of strict oncological surveillance. Conversely, patients #29 and #19 have a mild phenotype and carry, respectively, the unreported homozygous c.3265G>T and c.3054A>G variants, both sparing the helicase domain. Finally, despite matching several criteria for RTS clinical diagnosis, patient #38 is heterozygous for c.2412_2414del; no pathogenic CNVs out of those evidenced by high-resolution CGH-array, emerged as contributors to her phenotype.

[Hand eczema in children. Clinical and epidemiological study of the population referred to a tertiary hospital]. / Eccema de manos en niños. Estudio clínico-epidemiológico de la población remitida a un hospital terciario.

INTRODUCTION: Hand eczema is a frequent disease in adults. Diagnosing the cause of hand eczema is difficult due to different classifications. There is lack of evidence on hand eczema and its causes in children. MATERIAL AND METHOD: A total of 389 children between 0 and 16 years were identified between 1996 and 2016, from whom 42 (10.8%) with exclusively hand eczema were selected. In all cases a standard battery of epicutaneous patch tests was performed, as well as additional batteries depending on the clinical suspicion. The clinical and epidemiological features of these children were recorded and compared against children with eczema in other locations. RESULTS: The 42 children with hand eczema included 25 (60.5%) girls, and 17 (40.5%) boys, with a mean age of 10.6 +- 3.9 years, and did not differ from that of children with eczema in other locations. The definitive diagnosis after patch-testing was Atopic Dermatitis in 15 cases, Allergic Contact Dermatitis in 14 patients, Endogenous Vesiculous Eczema in 6 cases, Endogenous Hyperkeratotic Eczema in 5 cases, and Irritant Contact Dermatitis in 2 cases. The most frequent allergens detected were thiomersal (9 cases), nickel (5 cases), mercury (5 cases), and cobalt (4 cases). CONCLUSION: Hand eczema is a common condition in children. The most common cause is atopic dermatitis, although cases of allergic contact dermatitis manifesting as hand eczema are not uncommon. Any child with eczema of hands in whom an allergic cause is suspected should be referred for patch- testing.

Acneiform eruption induced by vedolizumab.

The development of new biological drugs for the treatment of advanced oncological processes or severe inflammatory diseases brings with it the appearance of new adverse effects. Vedolizumab, an α4ß7 integrin inhibitor antibody, is approved for induction and maintenance therapy in both Crohn disease and ulcerative colitis. We report a case of severe acneiform eruption induced by vedolizumab in a 17-year-old woman with ulcerative colitis.

Presentaciones atípicas de xantogranuloma juvenil / Atypical presentations of juvenile xanthogranuloma

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