Darier disease: the use of dermoscopy in monitoring acitretin treatment

Abstract Darier disease is an uncommon autosomal dominant inherited disease, caused by a mutation in the ATP2A2 gene. The clinical findings are hyperkeratotic papules on the trunk, scalp, face, and neck, maceration of intertriginous areas, palmar pits, whitish papules on the oral mucosa and nail abnormalities. The main histopathologic findings are acantholysis and dyskeratotic keratinocytes. Dermatoscopic features are comedo-like openings with a central polygonal yellowish/brownish structure, surrounded by a whitish halo. First-line treatment includes acitretin. Five reports have been published describing Darier disease dermatoscopic findings. Herein, we report for the first time a patient under acitretin treatment and dermatoscopic follow-up.

Terapias cuádruples son superiores a terapia triple estándar en primera línea de erradicación de Helicobacter pylori en Chile / Quadruple therapies are superior to standard triple therapy for Helicobacter pylori first-line eradication in Chile

Introducción: La infección por Helicobacter pylori afecta aproximadamente al 70% de la población chilena. Es un problema de salud pública cuyo tratamiento de erradicación forma parte de políticas públicas en Chile. Objetivos: Caracterizar los esquemas de erradicación de primera línea de H. pylori más utilizados en nuestro medio y evaluar su eficacia. Métodos: Se realizó un estudio observacional retrospectivo donde, en pacientes con infección por H. pylori certificada, se evaluó el esquema de erradicación indicado por el médico tratante, su eficacia, adherencia y efectos adversos. Resultados: Se analizaron 242 pacientes y 4 esquemas de erradicación; terapia triple estándar, terapia dual, concomitante y cuadriterapia con bismuto. Se observaron tasas de erradicación de 81,9% (IC 95% 74,44-87,63), 88,5% (IC 95% 73,13-95,67), 93,7% (IC 95% 78,07-98,44) y 97,6% (IC 95% 84,81-99,67) respectivamente, siendo más eficaces la terapia concomitante (RR: 1,14; IC 95% 1,01-1,29; p=0,028) y cuadriterapia con bismuto (RR: 1,19; IC 95% 1,09-1,31; p<0,001) que la triple terapia estándar. La tasa de efectos adversos reportados fue de 58,5% (IC 95% 50,66-65,92), 35,4% (IC 95% 24,6-48,11), 22,9% (IC 95% 11,81-37,14) y 63,4% (IC 95% 47,8-76,64), para la terapia triple estándar, dual, concomitante, y cuádruple con bismuto, respectivamente. La terapia dual y concomitante tuvieron menos efectos adversos en comparación con la terapia estándar. Conclusiones: Las cuadriterapias son superiores a la triple terapia estándar por lo que deberían ser consideradas como tratamiento de primera línea en Chile. La terapia dual es promisoria. Más estudios serán requeridos para determinar qué esquemas son más costo-efectivos.(AU)

Introduction: Helicobacter pylori infection affects approximately 70% of the Chilean population. It is a public health problem whose eradication treatment is part of the explicit health guarantees in Chile. Objectives: Characterize the most widely used H. pylori first-line eradication therapies in our environment and evaluate their efficacy. Methods: A retrospective observational study was carried out where, in patients with certified H. pylori infection, the eradication therapy indicated by the treating physician, its efficacy, adherence and adverse effects, in addition to the eradication certification method used, were evaluated. Results: 242 patients and 4 main therapies were analyzed: standard triple therapy, dual therapy, concomitant therapy, and bismuth quadruple therapy. Eradication rates of 81.9% (95% CI 74.44–87.63), 88.5% (95% CI 73.13–95.67), 93.7% (95% CI 78.07–98.44) and 97.6% (95% CI 84.81–99.67) were observed respectively, with concomitant therapy (RR: 1.14; 95% CI 1.01–1.29; p=.028) and quadruple therapy with bismuth (RR: 1.19; 95% CI 1.09–1.31; p<.001) being significantly more effective than standard triple therapy. Regarding the rate of reported adverse effects, it was 58.5% (95% CI 50.66–65.92), 35.4% (95% CI 24.6–48.11), 22.9% (95% CI 81–37.14) and 63.4% (95% CI 47.8–76.64), having the dual and concomitant therapy significantly fewer adverse effects compared with standard therapy. Conclusions: Quadruple therapies are superior to standard triple therapy and should be considered as first-line treatment in Chile. Dual therapy is promising. More studies will be required to determine which therapies are most cost-effective.(AU)

Darier disease: the use of dermoscopy in monitoring acitretin treatment.

Darier disease is an uncommon autosomal dominant inherited disease, caused by a mutation in the ATP2A2 gene. The clinical findings are hyperkeratotic papules on the trunk, scalp, face, and neck, maceration of intertriginous areas, palmar pits, whitish papules on the oral mucosa and nail abnormalities. The main histopathologic findings are acantholysis and dyskeratotic keratinocytes. Dermatoscopic features are comedo-like openings with a central polygonal yellowish/brownish structure, surrounded by a whitish halo. First-line treatment includes acitretin. Five reports have been published describing Darier disease dermatoscopic findings. Herein, we report for the first time a patient under acitretin treatment and dermatoscopic follow-up.

Current knowledge of immunosuppression as a risk factor for skin cancer development.

This review outlines our current understanding of the relationship between immunosuppression and skin cancer. Primary immunodeficiencies increase the incidence of skin cancer, but due to their low frequency, the establishment of accurate risk ratios remains lacking. Regarding secondary immunosuppression, available data demonstrate a significant increase of skin cancer in solid organ recipients, being the most common malignancy of this population. Immunosuppressive drugs have an important role in these patients, with an impact related to both the cumulative dose and the type of regimen used. The association of skin cancer and immunosuppressive drugs in non-transplant patients is conflictive for most of the drugs except for azathioprine. Many cancers lead to secondary immunosuppression as a mechanism for tumor growth and advancement, increasing the risk of progression of the primary tumor. Novel insights related with tumorigenesis and immune-escape mechanisms have led to promising new treatments in melanoma and squamous cell carcinoma.

Quadruple therapies are superior to standard triple therapy for Helicobacter pylori first-line eradication in Chile. / Terapias cuádruples son superiores a terapia triple estándar en primera línea de erradicación de Helicobacter pylori en Chile.

INTRODUCTION: Helicobacter pylori infection affects approximately 70% of the Chilean population. It is a public health problem whose eradication treatment is part of the explicit health guarantees in Chile. OBJECTIVES: Characterize the most widely used H. pylori first-line eradication therapies in our environment and evaluate their efficacy. METHODS: A retrospective observational study was carried out where, in patients with certified H. pylori infection, the eradication therapy indicated by the treating physician, its efficacy, adherence and adverse effects, in addition to the eradication certification method used, were evaluated. RESULTS: 242 patients and 4 main therapies were analyzed: standard triple therapy, dual therapy, concomitant therapy, and bismuth quadruple therapy. Eradication rates of 81.9% (95% CI 74.44-87.63), 88.5% (95% CI 73.13-95.67), 93.7% (95% CI 78.07-98.44) and 97.6% (95% CI 84.81-99.67) were observed respectively, with concomitant therapy (RR: 1.14; 95% CI 1.01-1.29; p=.028) and quadruple therapy with bismuth (RR: 1.19; 95% CI 1.09-1.31; p<.001) being significantly more effective than standard triple therapy. Regarding the rate of reported adverse effects, it was 58.5% (95% CI 50.66-65.92), 35.4% (95% CI 24.6-48.11), 22.9% (95% CI 81-37.14) and 63.4% (95% CI 47.8-76.64), having the dual and concomitant therapy significantly fewer adverse effects compared with standard therapy. CONCLUSIONS: Quadruple therapies are superior to standard triple therapy and should be considered as first-line treatment in Chile. Dual therapy is promising. More studies will be required to determine which therapies are most cost-effective.

Bullous systemic lupus erythematosus: An uncommon manifestation in pediatric population. / Lupus eritematoso sistémico buloso: Una manifestación infrecuente en población pediátrica.

INTRODUCTION: Bullous systemic lupus erythematosus (BSLE) is an autoimmune subepidermal blistering disease se condary to the presence of autoantibodies against type VII collagen of the basement membrane zone. It is considered a variant of Systemic Lupus Erythematosus (SLE) and is uncommon in the pediatric population. OBJECTIVE: To describe the case of a pediatric patient with a bullous eruption compati ble with BSLE. CLINICAL CASE: A 16-year-old female patient of Mapuche descent with history of SLE diagnosed at age 10, undergoing treatment. She consulted due to a six-week history of a generalized bullous eruption with no systemic symptoms. Biopsy for histology and direct immunofluorescence (DIF) confirmed the diagnosis of BSLE. The patient responded favorably to dapsone 100 mg/day (associated with her baseline treatment), without new reactivations after 8 years of follow-up. Con clusion: BSLE is an infrequent manifestation of SLE. The clinical presentation is similar to other bullous dermatoses, but the histopathology and DIF in correlation with the presence of SLE confirm the diagnosis. Although indigenous ancestry is associated with SLE high-risk alleles, studies regarding the association of BSLE in this ethnic group are still lacking.

Multifocal capillary malformation with segmental distribution and central atrophy: A case in a 12-year-old girl.

We present a 12-year-old girl with multiple geographic capillary malformations in a segmental distribution over the left trunk and arm that were present at birth and evolved over years with ulceration, atrophy, and subsequent scarring. Our case is clinically consistent with the recently described entity "multifocal capillary malformation with segmental distribution and central atrophy." To our knowledge, our patient is the oldest reported to date.