Bullous lupus as the first manifestation of systemic lupus erythematosus in the pediatric population: A diagnostic challenge in daily practice.

INTRODUCTION: In the GLADEL cohort, the bullous lupus (BSLE) prevalence was 0.41%. However, literature on pediatric BSLE is scarce. This study described the clinical, histological, and immunological characteristics and the treatment response in a series of children with BSLE as the first clinical manifestation of pediatric SLE. METHODS: The clinical, histological, and immunological characteristics of a series of 5 cases of BSLE between 2010-2019 from two reference centers in Colombia were analyzed. RESULTS: All cases had bullous lesions that resolved with residual hypopigmentation. One had a focal seizure, and another arthritis with thrombocytopenia. Two had transient proteinuria with normal urinalysis. Anti-nuclear antibody titers ranged from 1:160 to 1:2560, and four were anti-dsDNA (+). Five patients had anti-RNP antibodies, and four anti-Sm antibodies. All had low C3, and 80% low C4 counts; 80% had erythrocyte sedimentation rate (ESR) ≥20 mm/hour and 60% had C-reactive protein (CRP) ≥0.5 mg/dL. All patients responded to glucocorticoids and dapsone. Histology reports and direct immunofluorescence (DIF) test showed subepidermal blisters with neutrophils in the papillary dermis and linear deposits of Igs/complement proteins in 80% of the skin biopsies. IgG/IgM was present in 5 samples. IgA was positive in 60% and C3 in 80%. CONCLUSIONS: In this pediatric series, BSLE tends to have a monophasic behavior associated with neuropsychiatric, skeletal, and hematological involvement in 40% of the patients, and with good prognosis.

Papulonodular mucinosis in the primary onset of juvenile systemic lupus erythematosus: An unusual association / Mucinosis papulonodular en el inicio de lupus eritematoso sistêmico juvenil: asociación inusual

ABSTRACT Cutaneous mucinosis is a group of conditions characterized by the abnormal deposition of mucin in the skin. They can be primary, which in turn can be inflammatory-degenerative, and hamartomatous-neoplastic; or secondary. Papulonodular mucinosis is part of the group of dermal inflammatory-degenerative primary mucinosis. Its association with autoimmune connective tissue diseases has been described, especially with systemic lupus erythematosus, but it is considered an unusual manifestation of this disease. The clinical case is presented of an 11 year-old girl who, at the onset of systemic lupus erythematosus, presented with skin lesions for which the histopathological diagnosis corresponded to mucinosis.

RESUMEN Las mucinosis cutáneas son un grupo de condiciones caracterizadas por el depósito anormal de mucina en la piel. Pueden ser primarias, que a su vez pueden ser inflamatorias-degenerativas (dérmicas o foliculares) y hamartomatosas-neoplásicas; o secundarias. La mucinosis papulonodular forma parte de las mucinosis primarias inflamatorias-degenerativas dérmicas. Se ha descrito su asociación con enfermedades autoinmunes del tejido conectivo, especialmente con el lupus eritematoso sistémico, pero se considera una manifestación inusual de esta enfermedad. Se presenta el caso clínico de una niña de 11 años, quien al inicio del lupus eritematoso sistémico presentaba lesiones en la piel cuyo diagnóstico histopatológico correspondió a mucinosis.

Fatty Liver Disease and Gut Microbiota: A Comprehensive Update.

Nonalcoholic fatty liver disease (NAFLD) is the accumulation of fat in the liver in the absence of secondary causes. NAFLD is a multifactorial disease that results from the interaction of genetic predisposition and metabolic, inflammatory and environmental factors. Among these factors, dysregulation of gut microbiome has been linked to the development of fatty liver disease. The microbiome composition can be modified by dietary habits leading to gut microbiome dysbiosis, especially when a diet is rich in saturated fats, animal products and fructose sugars. Different species of bacteria in the gut metabolize nutrients differently, triggering different pathways that contribute to the accumulation of fat within the liver and triggering inflammatory cascades that promote liver damage. In this review, we summarize the current understanding of the roles of gut microbiota in mediating NAFLD development and discuss possible gut microbiota-targeted therapies for NAFLD. We summarize experimental and clinical evidence, and draw conclusions on the therapeutic potential of manipulating gut microbiota to decrease the incidence and prevalence of fatty liver disease.

Transfusion-free management of gastrointestinal bleeding: the experience of a bloodless institute.

GOALS: The Institute for Patient Blood Management and Bloodless Medicine at the Englewood Hospital has considerable experience in managing patients with gastrointestinal bleeding who do not accept blood-derived products. We present our data and experience over the last 8 years in managing such patients. BACKGROUND: There is paucity of data on management and outcomes of gastrointestinal bleeding in patients who do not accept blood-derived products. STUDY: We performed a retrospective study of patients from 2003 to 2011 presenting with gastrointestinal bleeding who do not accept blood-derived products. Inclusion criteria were either overt bleeding with a presenting hemoglobin (Hb) of <12 g/dL or a decrease in Hb of >1.5 g/dL. RESULTS: Ninety-six patients who met the inclusion criteria were included. Forty-one upper and 48 lower gastrointestinal bleeding sources were identified. Mean Hb was 8.8 g/dL and mean nadir was 6.9 g/dL. Among 37 patients (80.5%) with Hb ≤6.0 g/dL, 30 (81%) survived. Four of 7 patients (57%) with a Hb <3 g/dL survived. The overall mortality rate was 10.4%. In unadjusted logistic regression models, age [1.06 (1.01-1.12 y)], admission to ICU [6.37(1.27-31.9)], and anticoagulation use [6.95 (1.57-30.6)] were associated with increased mortality. Initial Hb [0.68 (0.51-0.92)] and nadir Hb [0.48 (0.29-0.78)] inversely predicted mortality. CONCLUSIONS: These results suggest that transfusion-free management of gastrointestinal hemorrhage can be effective with mortality comparable with the general population accepting medically indicated transfusion. Management of these patients is challenging and requires a dedicated multidisciplinary team approach knowledgeable in techniques of blood conservation.