Efficacy and Safety of Dupilumab for the Treatment of Severe Atopic Dermatitis in Clinical Practice: A Single Center Experience. / [Artículo traducido]Eficacia y seguridad de dupilumab para el tratamiento de la dermatitis atópica grave en la práctica clínica: experiencia en un centro terciario.

BACKGROUND: Dupilumab is a new targeted therapy for severe atopic dermatitis (AD) with limited real-world evidence. OBJECTIVE: Explore our experience with dupilumab for AD in clinical practice at a tertiary care center. MATERIAL AND METHOD: Unicentric observational retrospective study including adult and pediatric patients with severe AD receiving dupilumab between December 2017 and December 2021. The Eczema Area and Severity Index (EASI) score, Pruritus Numerical Rating Scale (P-NRS) and Sleep disturbance Numerical Rating Scale (S-NRS) were recovered to assess severity and response. RESULTS: Fifty-nine patients received dupilumab: 52, 48, 26 and 13 patients reached 6, 12, 24 and 36 months of treatment, respectively. The EASI-75 response rates were 94.2%, 95.8%, 92.3% and 100% at months 6, 12, 24 and 36. The EASI-90 response rates were 63.5%, 72.9%, 84.6% and 92.3% at months 6, 12, 24 and 36. The EASI <7 response rates were 92.3%, 91.7%, 88.5% and 100% at months 6, 12, 24 and 36. The P-NRS ≥4 reduction rates were 86%, 87.5%, 92.3% and 100% at months 6, 12, 24 and 36. The S-NRS ≥4 reduction rates were 82.7%, 85.4%, 100% and 100% at months 6, 12, 24 and 36. Adverse events were mild and occurred in 20.3% of patients, all of them adults. CONCLUSION: Our findings support dupilumab's favorable efficacy and tolerability profile in clinical practice. Dupilumab offers a rapid and sustained response, regardless of combined therapy. Longer follow-ups are still required to adequately assess its performance.

Efficacy and Safety of Dupilumab for the Treatment of Severe Atopic Dermatitis in Clinical Practice: A Single Center Experience.

BACKGROUND: Dupilumab is a new targeted therapy for severe atopic dermatitis (AD) with limited real-world evidence. OBJECTIVE: Explore our experience with dupilumab for AD in clinical practice at a tertiary care center. MATERIAL AND METHOD: Unicentric observational retrospective study including adult and pediatric patients with severe AD receiving dupilumab between December 2017 and December 2021. The Eczema Area and Severity Index (EASI) score, Pruritus Numerical Rating Scale (P-NRS) and Sleep disturbance Numerical Rating Scale (S-NRS) were recovered to assess severity and response. RESULTS: Fifty-nine patients received dupilumab: 52, 48, 26 and 13 patients reached 6, 12, 24 and 36 months of treatment, respectively. The EASI-75 response rates were 94.2%, 95.8%, 92.3% and 100% at months 6, 12, 24 and 36. The EASI-90 response rates were 63.5%, 72.9%, 84.6% and 92.3% at months 6, 12, 24 and 36. The EASI <7 response rates were 92.3%, 91.7%, 88.5% and 100% at months 6, 12, 24 and 36. The P-NRS ≥4 reduction rates were 86%, 87.5%, 92.3% and 100% at months 6, 12, 24 and 36. The S-NRS ≥4 reduction rates were 82.7%, 85.4%, 100% and 100% at months 6, 12, 24 and 36. Adverse events were mild and occurred in 20.3% of patients, all of them adults. CONCLUSION: Our findings support dupilumab's favorable efficacy and tolerability profile in clinical practice. Dupilumab offers a rapid and sustained response, regardless of combined therapy. Longer follow-ups are still required to adequately assess its performance.

Pseudoxanthoma elasticum in childhood in patient with ß-thalassaemia. / Pseudoxantoma elástico infantil en paciente con ß-talasemia.

Pseudoxanthoma elasticum (PXE) is a rare genetic disorder characterised by elastic tissue alterations and caused by mutations in a single gene, ABCC6, on chromosome 16p that includes manifestations that are predominantly cutaneous, ocular and cardiovascular. PXE-like lesions in association with ß-thalassemia have previously been reported in the literature in patients with ß-thalassaemia intermediate and major, being clinically indistinguishable from classic PXE. The case is presented of a 10-year-old boy with ß-thalassaemia minor and characteristic lesions of PXE. It is worth noting the benefit of multimodal imaging in the diagnosis and monitoring of the lesions.

Comparison of Phototherapy in Pediatric and Adult Patients. / Experiencia en el uso de fototerapia en pacientes pediátricos y comparación de esta técnica frente a pacientes adultos.

INTRODUCTION: Phototherapy involves the use of UV radiation to treat different dermatologic diseases. Its efficacy and safety have been thoroughly established in adults and some publications indicate that it is also an effective and safe treatment in pediatric patients with refractory skin diseases. MATERIAL AND METHODS: Retrospective study that included all patients under 17 years of age and 122 randomly selected adults who received phototherapy in our department between 2002 and 2017. RESULTS: Ninety-eight pediatric patients (61% girls and 39% boys) with a mean age of 10.5 years received phototherapy. The 3 most frequently treated diseases were psoriasis (48% of patients), vitiligo (17%), and atopic dermatitis (16%). Eighty-six percent of the patients received phototherapy with narrowband UV-B, whereas 7% received phototherapy with psoralen and UV-A (PUVA). No statistically significant differences were found in terms of dosage, duration, or number of sessions compared to the adult population treated with narrowband UV-B therapy or PUVA. A complete response was achieved in 35% of the pediatric patients and no differences were found with respect to the adults. Only 16% of the children showed adverse effects, mostly in the form of mild erythema. We found greater adherence to treatment in the pediatric patients than in the adult patients (P<.05). CONCLUSIONS: Narrowband UV-B therapy and PUVA appear to be safe and effective in children and can be administered using the same treatment protocols as those used in adults. Adherence to treatment is greater in children than in adult patients.

Association Between Bullous Pemphigoid and Dipeptidyl Peptidase 4 Inhibitors: A Retrospective Cohort Study. / Asociación entre penfigoide ampolloso e inhibidores de la dipeptidilpeptidasa-4: estudio de cohortes retrospectivo.

BACKGROUND: The association between dipeptidyl peptidase 4 inhibitors (DPP-4i) and bullous pemphigoid (BP) has been demonstrated in several studies. The main aim of this study was to estimate the use of DPP-4i treatment in patients diagnosed with BP in our setting. METHODS: We selected patients histologically diagnosed with BP in our department between October 2015 and October 2018 and performed a retrospective chart review to assess clinical and epidemiological data and direct immunofluorescence (DIF) patterns. RESULTS: Of the 70 patients diagnosed with BP during the study period, 50% were diabetic and 88.57% of these were being treated with a DPP-4i when diagnosed with BP. The most common DPP-4i was linagliptin (used in 18.6% of patients), followed by vildagliptin (17.1%). The median latency period between initiation of DPP-4i treatment and diagnosis of BP was 27.5 months for all treatments, 16 months for linagliptin, and 39 months for vildagliptin (log rank < 0.01). A negative DIF result was significantly more common in patients not being treated with a DPP-4i. The DIF pattern most strongly (and significantly) associated with DPP-4i treatment was linear immunoglobulin G deposits along the dermal-epidermal junction. DPP-4i treatment was withdrawn in 87% of patients and 96% of these achieved a complete response. CONCLUSIONS: DPP-4i treatment is very common in patients with BP in our setting. The latency period between start of treatment and onset of BP seems to be shorter with linagliptin than with other types of gliptins. Patients receiving DPP-4i treatment may show different DIF patterns to those not receiving treatment.

Genital Infection With Herpes Simplex Virus Type 1 and Type 2 in Valencia, Spain: A Retrospective Observational Study. / Infecciones genitales por virus herpes tipo 1 y virus herpes tipo 2 en Valencia, España: estudio observacional retrospectivo.

INTRODUCTION AND OBJECTIVE: The epidemiology of genital herpes has changed in recent years with an increase in the incidence of herpes simplex virus type 1 (HSV-1) infection. The aim of this study was to analyze the clinical and epidemiological characteristics of patients diagnosed with genital herpes. MATERIAL AND METHODS: A retrospective observational study was designed. All patients diagnosed with genital herpes between January 2016 and January 2019 in a Sexually Transmitted Infections Unit (ITS) in Valencia, Spain, were included. RESULTS: We identified 895 STI diagnoses. Of these, 126 (14%) were genital herpes; 68 (54%) of these cases were in women and 58 (46%) in men. Diagnosis was confirmed by molecular detection of HSV DNA in 110 cases (87.3%). Of these, 52 were cases of HSV-1 infection (47.3%) and 58 were HSV-2 infection (52.7%). HSV-2 was more common in men (69.5%), while HSV-1 was more common in women (59.3%). In the subgroup of women, mean age at diagnosis was 26 years for HSV-1 and 34 years for HSV-2 (P=.015). Recurrent genital herpes rates were 13% for HSV-1 and 40% for HSV-2. CONCLUSIONS: There has been an increase in the number of cases of genital herpes caused by HSV-1 in our setting, with young women in particular being affected. This has important prognostic implications because genital herpes caused by HSV-1 is less likely to recur.

Pediatric Allergic Contact Dermatitis: Clinical and Epidemiological Study in a Tertiary Hospital. / Dermatitis alérgica de contacto pediátrica. Estudio clínico-epidemiológico en un hospital terciario.

BACKGROUND: Few epidemiological studies have investigated the incidence of allergic contact dermatitis in children. Underdiagnosis has been observed in some studies, with many cases in which the condition is not suspected clinically and patch tests are not performed. However, the prevalence of pediatric sensitization to allergens has been reported to be as high as 20%, and the diagnosis should therefore be contemplated as a possibility in this age group. MATERIAL AND METHODS: We performed a retrospective analysis of the skin allergy database of the Dermatology Department of Consorcio Hospital General Universitario de Valencia. Children between 0 and 16 years of age diagnosed with allergic contact dermatitis in the previous 15 years (between 2000 and 2015) were included in the analysis. Epidemiological (age, sex, history of atopy) and clinical (site of the lesions, allergen series applied, positive reactions, and their relevance) variables were gathered. RESULTS: Patch tests had been performed on 4,593 patients during the study period. Of these, 265 (6%) were children aged between 0 and 16 years. A positive reaction to at least one of the allergens tested was observed in 144 (54.3%) patients in that group. The allergens most frequently identified were the following (in decreasing order of frequency): thiomersal, cobalt chloride, colophony, paraphenylenediamine, potassium dichromate, mercury, and nickel. The sensitization was considered relevant in 177 (61.3%) cases. CONCLUSIONS: More than half of the children studied showed sensitization to 1 or more allergens, with a high percentage of relevant sensitizations. All children with a clinical suspicion of allergic contact dermatitis should be referred for patch testing. As no standardized test series have been developed for this age group, a high level of clinical suspicion and knowledge of the allergens most commonly involved are required when selecting the allergens to be tested.

Allergic contact dermatitis due to cosmetics: A clinical and epidemiological study in a tertiary hospital. / Dermatitis alérgica de contacto a cosméticos, estudio clínico-epidemiológico en un hospital terciario.

The incidence of allergic contact dermatitis (ACD) to cosmetics in the general population is rising with the increasing use of cosmetic products and their proliferation and diversification. The aims of this study were to determine the prevalence of ACD to cosmetics in our setting, analyze changes over time, describe the clinical and epidemiological features of this allergic reaction, and identify the allergens and cosmetics involved. We performed a prospective study at the skin allergy unit in Hospital General Universitario de Valencia in Spain between 2005 and 2013 and compared our findings with data collected retrospectively for the period 1996 to 2004. The 5419 patients who underwent patch testing during these 2 periods were included in the study. The mean prevalence of ACD to cosmetics increased from 9.8% in the first period (1996-2004) to 13.9% in the second period (2005-2013). A significant correlation was found between ACD to cosmetics and female sex but not atopy. Kathon CG (blend of methylchloroisothiazolinone and methylisothiazolinone), fragrances, and paraphenylenediamine were the most common causes of ACD to cosmetics during both study periods, and acrylates and sunscreens were identified as emerging allergens during the second period.

[Tufted angiomas in childhood: A series of 9 cases and a literature review]. / Angiomas en penacho en la infancia. Serie de 9 casos y revisión de la literatura.

INTRODUCTION: Tufted angioma (TA) is a rare benign vascular tumor that mostly appears during infancy or early childhood, although there are cases reported in adults. Clinical presentation and evolution of TA can vary. Histologically, it takes on a classic appearance of vascular tufts ("cannon ball" like appearance). PATIENTS AND METHODS: A retrospective observational study was conducted that included all patients diagnosed with TA at our center in the last 20 years. RESULTS: A series of 9 cases of tufted angioma in childhood are presented, 77.7% of which were congenital. This represents a frequency higher than previously described. Spontaneous regression was observed in 55.5% of the cases, and was more frequent in the congenital TA group. Unlike other TA series reported in the literature, a higher proportion of patients with spontaneous regression was observed in this series, with a higher prevalence in females (6 out of 9 children) and predominantly located in the upper limbs. None of our patients had Kasabach-Merritt phenomenon. CONCLUSIONS: There are many ways of treating TA, but none are uniformly effective. Given the high rate of spontaneous regression in congenital or early TA, we suggest that, in the absence of other complications, monitoring would be a good option for management.

[Pyoderma gangrenosum associated with hidradenitis suppurativa: a case report and review of the literature]. / Pioderma gangrenoso asociado a hidrosadenitis supurativa: aportación de un caso y revisión de la literatura.

Pyoderma gangrenosum is an inflammatory disease that has been found to be associated with many systemic illnesses. The case presented here is of a man with a 20-year history of hidradenitis suppurativa who developed pyoderma gangrenosum. The pyoderma lesions appeared as a single outbreak which resolved totally after immunosuppressive treatment. This association has been reported only rarely in the literature. Furthermore, in the cases reported, no relationship was apparent between the activity of both diseases. In all cases the clinical course appeared independent, with no apparent overlap in inflammatory activity or response to the drugs administered.