The role of excimer light in dermatology: a review.

Excimer light is a subtype of NB-UVB that emits a 308 nm wavelength, and can provide targeted phototherapy treatment. The absorption of 308 nm light by skin cells leads to therapeutic response in various common and ultraviolet-responsive skin diseases, such as psoriasis and vitiligo, and photo-resistant skin diseases such as prurigo nodularis, localized scleroderma, genital lichen sclerosis, and granuloma annulare, cutaneous T-cell lymphomas, among others. Excimer light has few adverse reactions and overall is well tolerated by patients, furthermore, it can be performed in places that are difficult to access. This article aims to explain the therapeutic bases and applications of excimer light in current dermatology.

2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc. METHODS: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed. RESULTS: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud's phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found. CONCLUSION: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice.

Spontaneous pneumoperitoneum in the absence of visceral perforation in a patient with systemic sclerosis: a case report.

We present the case of a 44 year old woman with systemic sclerosis who presented with intense abdominal pain without signs of peritonitis. An abdominal computed tomography showed generalized intestinal dilation, intestinal pneumatosis and an extensive pneumoperitoneum. A diagnostic laparoscopy was performed but no perforation nor gastrointestinal leakage were found. Spontaneous pneumoperitoneum in patients with systemic sclerosis without visceral perforation is an extremely rare complication. Physicians must have a low threshold of suspicion for this entity when a patient with systemic sclerosis presents with spontaneous pneumoperitoneum in the absence of peritoneal signs.

Presentamos el caso de una mujer de 44 años con diagnóstico de esclerosis sistémica, quien presentó dolor abdominal intenso sin datos de irritación peritoneal. Una tomografía computarizada de abdomen mostró dilatación generalizada de asas intestinales, neumatosis intestinal y neumoperitoneo extenso, por lo cual se realizó una laparoscopía diagnóstica, sin encontrar sitio de perforación. El neumoperitoneo espontáneo en pacientes con esclerodermia sin evidencia de perforación visceral es una complicación extremadamente rara. El médico deberá mantener un alto índice de sospecha para esta condición ante un paciente con esclerosis sistémica que se presente con un neumoperitoneo espontáneo sin datos de irritación peritoneal.

Diffuse Gastrointestinal Motor Compromise in Patients with Scleroderma: Utility of Minimally Invasive Techniques.

BACKGROUND: Scleroderma is a systemic inflammatory disorder that can compromise the gastrointestinal tract in up to 90% of patients. AIM: The purpose of this work is to characterize esophageal, gastric, and intestinal compromise in patients with scleroderma by means of minimally invasive methods and its association with symptoms and severity of their rheumatological condition. METHODS: Patients with systemic sclerosis were recruited according to the criteria of the American College of Rheumatology. The study of digestive involvement was carried out on four consecutive days: esophageal manometry was performed on the first day, intestinal manometry on the second day, surface electrogastrography on the third, and hydrogen breath test on the fourth. The Mann-Whitney test was used for quantitative variables and the chi-squared test for categorical variables (p < 0.05). RESULTS: A total of 30 patients were included, with an average age of 52.7 years and 93% women. Average disease evolution duration was 6.5 years, 70% with limited variety. Rodnan averaged 12 points, being higher in the diffuse variety. The main symptom was heartburn, followed by abdominal distension, with no differences between subtypes except for diffuse nausea; 80% had intestinal manometric compromise, 76% esophageal manometric compromise, and 30% electrogastrographic compromise. Bacterial overgrowth was evidenced in two-thirds (66%) of the patients, and 23% of the patients had simultaneous esophageal, gastric, and intestinal involvement, which correlated with greater skin involvement but not with gastrointestinal symptoms. CONCLUSIONS: Gastrointestinal involvement in patients with scleroderma is frequent and is observed regardless of the symptoms and clinical characteristics of the latter, except for skin involvement.

Ultrasound assessment of diaphragm and quadriceps muscles and its relationship with handgrip and respiratory muscle strength in patients with systemic sclerosis: a cross-sectional study.

BACKGROUND: Muscle dysfunction may cause disability and reduce the quality of life of patients with systemic sclerosis (SSc) when compared to healthy individuals. However, the literature on the topic is scarce and uses several criteria for assessing muscle dysfunction in this population. OBJECTIVES: To compare diaphragm and quadriceps muscle thickness, diaphragm mobility, and handgrip strength between patients with SSc and healthy individuals. METHOD: This cross-sectional study included 16 patients with SSc and 16 self-reported healthy individuals matched for age. We assessed quadriceps and diaphragm thickness and diaphragmatic mobility (ultrasound), handgrip strength (hand-held dynamometer), and respiratory muscle strength (manovacuometer). Patients also responded to the Health Assessment Questionnaire Disability Index and the International Physical Activity Questionnaire. RESULTS: Patients with SSc presented lower quadriceps thickness (p < 0.0001), diaphragmatic mobility (p = 0.01), handgrip (p < 0.0001), and respiratory muscle strength (p < 0.0001) than healthy individuals. A moderate positive correlation was observed between handgrip strength and quadriceps thickness in patients with SSc (rho = 0.576; p = 0.02). CONCLUSIONS: Patients with SSc presented reduced quadriceps thickness, diaphragmatic mobility, handgrip, and respiratory muscle strength when compared to healthy individuals Also, handgrip strength was correlated with quadriceps thickness in patients with SSc, suggesting that loss of muscle mass accompanies loss of peripheral muscle strength group of patients. Key Points • SSc patients presented reduced quadriceps thickness and diaphragmatic mobility • SSc patients have reduced handgrip and respiratory muscle strength • Lower handgrip muscle strength correlated with lower quadriceps thickness.

Quality of sleep in individuals with systemic sclerosis and its correlation with functional disability and quality of life: a cross-sectional study

SUMMARY OBJECTIVE: This study aimed to evaluate the quality of sleep in individuals with systemic sclerosis and its correlation with the quality of life and disability. METHODS: This is a cross-sectional study, carried out in a tertiary service of a university hospital. Inclusion criteria were diagnosis of systemic sclerosis according to the criteria of the American College of Rheumatology/European League Against Rheumatism 2013 or the preliminary criteria of the American College of Rheumatology 1980, age ≥ 18 years; regularly monitored at the outpatient clinic of rheumatology. Clinical and demographic data of the patients were obtained through a structured interview and evaluation of the medical records. Sleep quality was assessed using the Pittsburgh Sleep Quality Index questionnaire, daytime sleepiness using the Epworth Sleepiness Scale, quality of life using 12-item short-form health survey, and disability using the scleroderma health assessment questionnaire. RESULTS: A total of 50 patients with systemic sclerosis were included, with 92% female, mean age 48.9 years, mean disease duration 8.9 years, and 60% limited cutaneous form. Most systemic sclerosis patients (84%) have poor sleep quality and 20% have excessive daytime sleepiness. There was a significant negative correlation between Pittsburgh Sleep Quality Index and the physical and mental components of the 12-item short-form health survey (r=-0.42, p=0.003 and r=-0.43, p=0.002, respectively) and a positive correlation with the scleroderma health assessment questionnaire (r=0.52, p=<0.001). CONCLUSION: This study showed that poor sleep quality is a very common finding among systemic sclerosis patients, and it negatively affects both the quality of life and the degree of disability.

Connective tissue disease-associated interstitial lung disease / Doença pulmonar intersticial associada a doença do tecido conjuntivo

ABSTRACT Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a group of systemic autoimmune disorders characterized by immune-mediated organ dysfunction. Systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren's syndrome are the most common CTDs that present with pulmonary involvement, as well as with interstitial pneumonia with autoimmune features. The frequency of CTD-ILD varies according to the type of CTD, but the overall incidence is 15%, causing an important impact on morbidity and mortality. The decision of which CTD patient should be investigated for ILD is unclear for many CTDs. Besides that, the clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. A significant proportion of patients will present with a more severe and progressive disease, and, for those, immunosuppression with corticosteroids and cytotoxic medications are the mainstay of pharmacological treatment. In this review, we summarized the approach to diagnosis and treatment of CTD-ILD, highlighting recent advances in therapeutics for the various forms of CTD.

RESUMO Doença pulmonar intersticial associada à doença do tecido conjuntivo (DPI-DTC) representa um grupo de distúrbios autoimunes sistêmicos caracterizados por disfunção de órgãos imunomediada. Esclerose sistêmica, artrite reumatoide, miosite inflamatória idiopática e síndrome de Sjögren são as DTC mais comuns que apresentam acometimento pulmonar, bem como pneumonia intersticial com achados autoimunes. A frequência de DPI-DTC varia de acordo com o tipo de DTC, mas a incidência total é de 15%, causando um impacto importante na morbidade e mortalidade. A decisão sobre qual paciente com DTC deve ser investigado para DPI não é clara para muitas DTC. Além disso, o espectro clínico pode variar desde achados assintomáticos em exames de imagem até insuficiência respiratória e morte. Parte significativa dos pacientes apresentará doença mais grave e progressiva, e, para esses pacientes, imunossupressão com corticosteroides e medicamentos citotóxicos são a base do tratamento farmacológico. Nesta revisão, resumimos a abordagem do diagnóstico e tratamento de DPI-DTC, destacando os recentes avanços na terapêutica para as diversas formas de DTC.

Males and females with scleroderma: A comparative study in a Brazilian sample.

Objectives: This study aimed to evaluate the clinical and serological profile in systemic sclerosis (SSc) by comparing females and males. Patients and methods: This retrospective study was conducted with 215 SSc patients (193 females, 22 males; mean age: 50.1±14.5 years; range, 16 to 88 years) between September 2005 and September 2020. Disease severity was calculated by the Medsger severity score. Males and females were compared for clinical and serological markers. Results: Females more frequently had esophageal involvement (p=0.003), telangiectasias (p=0.03), and antinuclear antibodies (p=0.04). Males more frequently had fingertip scars (p=0.03), digital ulcers (p=0.006), and a worse median Medsger severity score (6 in males vs. 4 in females, p=0.05). Conclusion: In the studied sample, males had more severe disease than females with greater repercussions in periferic circulatory system.

Esclerodermia y alteraciones esofágicas documentadas por manometría / Scleroderma and Esophageal Alterations Documented by Manometry

Introducción: Las manifestaciones gastrointestinales y en especial la disfunción esofágica son frecuentes en pacientes con diagnóstico de esclerosis sistémica. Objetivos: Determinar los hallazgos manométricos en el esófago de pacientes con esclerosis sistémica. Métodos: Se realizó un estudio descriptivo de corte transversal, en 86 pacientes con diagnóstico de esclerosis sistémica que fueron atendidos en el Servicio de Reumatología del Hospital Hermanos Ameijeiras, en el período comprendido de enero de 2020 a diciembre de 2021. Resultados: La edad media fue de 49,5 ± 15,3 años, (94,3 por ciento) en el sexo femenino. El (90,7 por ciento) tenía trastornos de la motilidad esofágica, principalmente los trastornos mayores (58,1por ciento) y el esfínter esofágico corto (62,8 por ciento). La presencia de síntomas como la regurgitación, la pirosis y la disfagia se relacionaron de forma significativa en la mayoría de los parámetros manométricos. De igual forma, el tiempo de evolución de la enfermedad (10,1 ± 9,1 frente a 5,9 ± 5,9 años), el fenómeno de Raynaud (93,9 por ciento frente a 25,0 por ciento) y la esclerosis sistémica difusa (96,2 por ciento frente a 82,4 por ciento) fueron significativamente mayores en pacientes con trastornos de la motilidad esofágica. La edad y el sexo no mostraron una asociación significativa con las alteraciones manométricas. Conclusiones: Se concluye que los pacientes con esclerosis sistémica difusa, fenómeno de Raynaud, a partir de la presencia de los síntomas y de la evolución de la enfermedad tienen una elevada probabilidad de padecer trastornos de la motilidad esofágica(AU)

Introduction: Gastrointestinal manifestations, and especially esophageal dysfunction, are common in patients diagnosed with systemic sclerosis. Objectives: To determine the manometric findings in the esophagus of patients with systemic sclerosis. Methods: A descriptive cross-sectional study was carried out on 86 patients with a diagnosis of systemic sclerosis who were treated in the Rheumatology Service of Hermanos Ameijeiras Hospital from January 2020 to December 2021. Results: The mean age was 49.5 ± 15.3 years, (94.3percent) in females. 90.7percent had esophageal motility disorders, mainly major disorders (58.1percent) and 62.8percent had short esophageal sphincter. The presence of symptoms such as regurgitation, heartburn and dysphagia were significantly related to most manometric parameters. Similarly, the duration of the disease (10.1 ± 9.1 versus 5.9 ± 5.9 years), Raynaud's phenomenon (93.9percent versus 25.0percent) and sclerosis diffuse systemic (96.2percent vs. 82.4percent) were significantly higher in patients with esophageal motility disorders. Age and sex did not show significant association with manometric alterations. Conclusions: It is concluded that patients with diffuse systemic sclerosis, Raynaud's phenomenon, based on the presence of symptoms, and the evolution of the disease, have high probability of suffering from esophageal motility disorders(AU)

[Characteristics of patients with systemic sclerosis who develop interstitial lung disease]. / Características de los pacientes con esclerosis sistémica que desarrollan enfermedad pulmonar intersticial.

Background: Systemic sclerosis associated-interstitial lung disease (SSc-ILD) show a high mortality. The factors associated SSc-ILD have shown variability in different populations. There are few studies in Mexican mestizos. Objective: To analyze the epidemiological, clinical and paraclinical factors associated with SSc-ILD. Material and methods: Cross-sectional study, where patients > 18 years of age with a diagnosis of SSc according to EULAR/ACR 2013 criteria and diagnosis of ILD by forced vital capacity (FVC) < 70% and > 5% of affected lung area on tomography were included. The strength of association of the factors for ILD was measured by odds ratio (OR) with 95% confidence intervals (95% CI). The significant variables were analyzed by multiple logistic regression with adjustment. Results: Of the 80 patients with SSc, 38 (47.5%) had ILD. Risk factors such as smoking, higher activity index, lower FVC, anti-topoisomerase I antibodies, and protective factors such as a limited SSc, early capillaroscopic pattern, and anti-centromere antibodies (ACA) were associated with ILD. In the multivariate analysis, a higher activity index with OR 4.17, (95% CI 2.01-8.65) persisted as a risk factor, while ACA with OR 0.17, (95% CI 0.03-0.85) persisted as a protective factor associated with SSc-ILD. Conclusions: A higher index of activity and ACA persisted as factors associated with SSc-ILD.

Introducción: la enfermedad pulmonar intersticial (EPI) está relacionada a una mayor mortalidad en esclerosis sistémica (ES). Los factores asociados a su presentación han mostrado variabilidad en diferentes poblaciones. Pocos estudios se han realizado en mestizos mexicanos. Objetivo: analizar los factores clínicos y paraclínicos asociados a EPI en pacientes con ES. Material y métodos: estudio retrospectivo de casos y controles en el que se incluyeron pacientes > 18 años con diagnóstico de ES, según criterios EULAR/ACR 2013 y EPI por capacidad vital forzada (CVF) < 70% y > 5% de área pulmonar efectada en tomografía. La fuerza de asociación de los factores para EPI se midió por razón de momios (RM) con intervalos de confianza del 95% (IC95%). Las variables significativas se analizaron por regresión logística múltiple con ajuste. Resultados: de los 80 pacientes con ES, 38 (47.5%) tenían EPI. Factores de riesgo como tabaquismo, mayor índice de actividad, menor CVF, anticuerpos antitopoisomerasa I y factores protectores como variedad limitada de ES, patrón capilaroscópico temprano y anticuerpos anticentrómero estuvieron asociados a EPI. En el análisis multivariado un mayor índice de actividad con RM 4.17, (IC95%: 2.01-8.65) persistió como un factor de riesgo; en cambio, los anticuerpos anticentrómero con RM 0.17, (IC95%: 0.03-0.85) persistieron como un factor protector asociado a EPI. Conclusiones: un mayor índice de actividad y la presencia anticuerpos anticentrómero persistieron como factores asociados a la presencia de EPI en ES.

Nodular (keloidal) scleroderma.

Keloidal or nodular scleroderma (NS) is a variant of localized scleroderma (LS) frequently seen in patients with limited or diffuse systemic sclerosis (SSc). It presents as raised, firm plaques or nodules with extensive dermal fibrosis and hyalinized collagen bundles. We present a patient with SSc who presented with this rare entity.

Sub-oclusión intestinal y desnutrición severa en esclerosis sistémica sin esclerodermia

La esclerosis sistémica sin escleroderma es una forma no común de compromiso sistémico de esta enfermedad, con ausencia de manifestaciones cutáneas. Presentamos el caso de una mujer de 71 años con índice de masa corporal (IMC) en 16,9 kg/m2, con historia de hipotiroidismo y estreñimiento crónico, quien acude a emergencia por dolor y distensión abdominal, asociado a náuseas. Se realizó una tomografía abdomino-pélvica encontrándose cámara gástrica colapsada, asas intestinales con algunos segmentos distendidos, con signos de fecalización de asa delgada. La colonoscopía describió dolicocolon severo. Se observaron placas induradas en codo derecho y en ambos glúteos. Los exámenes auxiliares: ANA 1/640 patrón centromérico, anticuerpos anticentrómero = 227 U/mL (valor normal - VN: 0-25), Anti SCL-70 = 1,9 U/mL (VN 0-25) y anti RNP = 0,1 U/mL (VN 0-25). La capilaroscopía de borde ungueal a 200x mostró megacapilares. El diagnóstico clínico fue esclerosis sistémica sin esclerodermia. Se inició tratamiento con dieta rica en fibra y nutrición parenteral por 1 semana. La evolución de la paciente fue favorable.

Systemic sclerosis sine Scleroderma is an uncommon form of systemic compromise of this disease, in the abscense of cutaneous manifestations. We present the case of a 71 years-old woman, body mass index (BMI) in 16.9 kg/m2, with history of hypothyroidism and chronic constipation, who goes to the emergency with pain, abdominal bloating, and nausea. An abdominopelvic tomography was made, reporting a collapse of the gastric chamber, some segments of intestinal loops distended with signs of fecalization in the small intestine. A colonoscopy described severe dolicocolon. We observed indurated plaques in left elbow and in both buttocks. Lab tests: ANA 1/640 with anticentromeric pattern, anticentromere antibodies = 227 U/mL (0-25), Anti-SCL-70 = 1.9 U/mL (0-25), anti-RNP = 0.1 U/mL (0-25). Peri-ungueal cappilaroscopy 200x showed mega capillaries. Clinical diagnosis was systemic sclerosis sine scleroderma. We initiated treatment with a high-fiber diet and parenteral nutrition for a week. The patient evolution was favorable.

Color Doppler Ultrasound Assessment of Subclinical Activity With Scoring of Morphea.

BACKGROUND: Detection of activity in morphea is paramount for adequately managing the disease. Subclinical ultrasound involvement on inactive lesions or healthy skin areas adjacent to morphea has not been described to date. OBJECTIVES: The study aimed to detect morphea's subclinical activity by Color Doppler ultrasound not identified with the clinical scorings. MATERIALS & METHODS: This cross-sectional retrospective study was done from January 2014 to July 2019 in patients with a clinicopathological diagnosis of morphea. The modified Localized Scleroderma Skin Severity Index (mLoSSI) and The Ultrasound Morphea Activity Score (US-MAS) were used to correlate clinical and subclinical activity. RESULTS: A total of 36 patients met the inclusion criteria. 54% of cases presented subclinical activity in areas adjacent to the clinically active lesion, 23% in nonadjacent regions, and 23% demonstrated activity at a clinically inactive lesion site.100% of patients with morphea "en coup de sabre" involving the frontal region of the face concomitantly presented both subclinical activities of morphea on the frontal facial region and the scalp following the same axis.A positive relationship was observed between the degree of clinical activity measured by mLoSSI and US-MAS scoring.The main limitations of our study were the low number of patients and the inability to detect alterations < 0.1 mm. CONCLUSIONS: Subclinical activity is frequent in morphea, can extend beyond the lesional areas, including apparently noninvolved adjacent and distant corporal regions, and can be detected by color Doppler ultrasound.

Screening tool development for hand surgery referrals in systemic sclerosis.

BACKGROUND: Systemic Sclerosis (SSc) patients may need hand surgery. OBJECTIVE: To develop a screening tool for rheumatologists to identify potential candidates with systemic sclerosis for hand surgery, optimizing referrals. METHODS: A pilot cross-sectional study from January 2015 to December 2016. SAMPLE SIZE: 51 participants. INCLUSION CRITERIA: ≥ 18 years old, meeting the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc and hand impairment. DATA COLLECTED: age, sex, race, disease duration, SSc subtypes, vasodilator use, skin thickness, finger stiffness, presence of Digital Ulcers (DU) and/or calcinosis, presence of Raynaud's Phenomenon (RP) attacks, health status and disability, disease status, pain intensity and functional status of the hands. Data were analyzed by a multivariate logistic regression model. RESULTS: Fulfillment of surgical criteria: 68.8%. The surgical group had higher scores on the HAQ-DI (1.39 vs. 0.96, p = 0.032) and CHFS (25.0 vs. 12.0, p = 0.005) questionnaires, and a higher frequency of DU (91.43% vs. 18.75%, p < 0.0010), calcinosis (60.0% vs. 0.0%, p < 0.001), use of vasodilators (100.0% vs. 75.0%, p = 0.007) and digital stiffness (28.57% vs. 0.0%, p = 0.017). The presence of DU increased the chance of surgical indication by 46.2 times (ORIC 95% = 8.23 to 259.49). The statistical model showed good accuracy (86.3%, p < 0.001), sensitivity (91.4%), and specificity (81.2%). CONCLUSION: The presence of DU in SSc could be used as a screening feature for early identification and referral of potential candidates for hand surgery.

Coadministration of 3'5-dimaleamylbenzoic acid and quercetin decrease pulmonary fibrosis in a systemic sclerosis model.

Systemic sclerosis (SSc) is an autoimmune disease characterized by microvascular compromise and fibrosis. Pulmonary fibrosis, a prominent pulmonary complication in SSc, results in impaired lung function due to excessive accumulation of extracellular matrix components. This study aimed to investigate the effects of coadministration of 3'5-dimaleamylbenzoic acid (AD) and quercetin (Q) on key events in the development and maintenance of pulmonary fibrosis in a bleomycin (BLM)-induced SSc mouse model. The model was induced in CD1 mice through BLM administration using osmotic mini pumps. Subsequently, mice were treated with AD (6 mg/kg) plus Q (10 mg/kg) and sacrificed at 21 and 28 days post BLM administration. Histopathological analysis was performed by hematoxylin and eosin staining and Masson's trichrome staining. Immunohistochemistry was used to determine the expression of proliferation, proinflammatory, profibrotic and oxidative stress markers. The coadministration of AD and Q during the fibrotic phase of the BLM-induced SSc model led to attenuated histological alterations and pulmonary fibrosis, reflected in the recovery of alveolar spaces (30 %, p < 0.01) and decreased collagen deposits (50 %, p < 0.001). This effect was achieved by decreasing the expression of the proliferative markers cyclin D1 (87 %, p < 0.0001) and PCNA (43 %, p < 0.0001), inflammatory markers COX-2 (71 %, p < 0.0001) and iNOS (84 %, p < 0.0001), profibrotic markers α-SMA (80 %, p < 0.0001) and TGF-ß (81 %, p < 0.0001) and the lipid peroxidation marker 4-HNE (43 %, p < 0.01). The antifibrotic effect of this combined therapy is associated with the regulation of proliferation, inflammation and oxidative stress, mechanisms involved in the development and progression of the fibrotic process. Our novel therapeutic strategy is the first approach to propose the use of the combination of prooxidant and antioxidant compounds as a potential strategy for SSc-associated pulmonary fibrosis.

Atrofodermia Idiopática de Pasini y Pierini, un abordaje inusual / Idiopathic atrophoderma of Pasini and Pierini, an unusual approach

La atrofodermia idiopática de Pasini y Pierini es una entidad poco frecuente y de etiología aún no esclarecida, se presenta con una frecuencia hasta seis veces mayor en mujeres que en hombres y una posible asociación con la esclerodermia localizada (morfea). Paciente femenina de 30 años, quien consultó por una lesión asintomática de dos años de evolución en el glúteo izquierdo. En el examen físico se evidenció una placa ovalada, deprimida y acrómica en su centro, que mide cinco por diez centímetros. La paciente había sido tratada previamente con múltiples terapias tópicas sin obtener mejoría clínica. Se realizó la biopsia de piel que demostraba cambios mínimos en epidermis, homogenización y adelgazamiento de colágeno sin afección de anexos. Se hizo correlación con los hallazgos clínicos y se decidió iniciar tratamiento con esteroides intralesionales de alta potencia (acetónido de triamcinolona). Posterior a la administración de dos aplicaciones del medicamento, con cuatro semanas de diferencia entre ellas, se evidenció la resolución completa de la dermatosis. Un mes después de la última dosis la paciente no mostró recidivas

diopathic atrophoderma of Pasini and Pierini is a rare entity of unclear etiology, occurring as much as six times more frequently in women than in men, with a possible association with localized scleroderma (morphea). It is about a 30 years old woman who consulted with an asymptomatic lesion of two years of evolution on the left gluteal region. Physical examination revealed an oval plaque, depressed and acromic in its center, measuring five by ten centimeters. A 30 years old female patient who consulted about an asymptomatic lesion of two years of evolution on the left gluteal region. Physical examination revealed an oval plaque, depressed and acromic in its center, measuring five by ten centimeters. The patient was previously treated with multiple topical therapies without clinical improvement.Skin biopsy showed minimal changes in the epidermis, homogenization, and thinning of the collagen without adnexal involvement. After a correlation was made with the clinical findings, starting treatment with high-potency intralesional steroids (triamcinolone acetonide) was recommended. After administering two applications of the drug, four weeks apart, the complete resolution of the dermatosis was evidenced. One month after the last dose, the patient showed no recurrence

Oral health education for systemic sclerosis patients: A booklet report.

Purpose/objectives: Systemic sclerosis (SSc) is a rare chronic autoimmune disease characterized by vascular abnormalities and connective tissue disorders. In 2021, the multidisciplinary team of the university hospital recognized a demand for oral health information among SSc patients. This study aims to describe the development of an SSc oral health booklet and its validation by people with SSc. Methods: For the development of the booklet, the project creators employed the action research methodology involving ten stages. SSc patients evaluated the material by filling out a digital form. Results: One hundred and thirty-one people with SSc evaluated the booklet. It received an average score of 9.73 (SD: 0.80) in relevance, 9.82 (SD: 0.47) in explicitness, and 9.49 (SD: 0.93) in aesthetics. The final version was emailed to the participants and posted on social media. Innovation: This is the first booklet that deeply addresses these issues in Portuguese and constitutes a soft technology to instruct and assist patients with SSc. Conclusion: The production of high-quality educational materials on oral health for patients with SSc is necessary. The online distribution increased its reach and had great importance in the context of the COVID-19 pandemic.

Ultrasound Morphea Activity Scoring (US-MAS): Modified US-MAS.

Morphea, an autoimmune connective tissue disease that affects the skin, can be supported by color Doppler ultrasound in its diagnosis and assessment of activity. To date, there are no reliable laboratory parameters to track activity, and ultrasound presents a higher axial spatial resolution than magnetic resonance imaging and computed tomography, which is critical for studying the superficial layers. The quality of the ultrasonographic assessment of activity in morphea depends on the standardization and features of the acquisition of the anatomical data. We propose a detailed ultrasound morphea activity scoring called modified US-MAS (mUS-MAS) that could allow us to systematically register the cutaneous abnormalities in the corporal regions and their subregions. The selection of the scanning sites will depend on the corporal regions of involvement and their adjacent segments. Through systematic and sequential ultrasound data analysis, we propose that this scoring system can better support description and activity tracking accuracy.