ABSTRACT
Skin involvement in systemic lupus erythematosus (SLE) occurs in more than 75% of patients with this condition. Vesicles and blisters in lupus erythematosus (LE) may be present in SLE secondary to interface vacuolar changes in the epidermis, in discoid LE also secondary to vacuolar epidermal changes, and in bullous LE secondary to antibodies anti-collagen VII deposits with neutrophilic aggregates. In addition, blisters can occur due to the association of SLE with other autoimmune blistering diseases (e.g. bullous pemphigoid). BSLE is a rare blistering disease that mainly occurs in females (3040 years old), and less frequently in children and adolescents. The most common presentation is rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques. Preferential sites are: superior trunk, proximal superior limbs, and face (lips) with symmetrical distribution. Mucosal involvement is common on perioral, pharyngeal, laryngeal, and genital areas. The involvement of sun-exposed areas is not mandatory. The lesions usually progress with no scarring, but hypo or hyperchromia may be present. We report an 18-year-old female patient with blistering lesions at admission, who was diagnosed with BSLE. She was initially treated with systemic prednisone and hydroxychloroquine. Her condition evolved with relapsing lesions, which required the introduction of Dapsone. The authors emphasize the relevance of recognizing BSLEa rare presentation of SLEwhich may evolve with marked clinical presentation
Subject(s)
Humans , Female , Adolescent , Skin Diseases, Vesiculobullous , Lupus Erythematosus, Systemic/diagnosis , Blister , Rare DiseasesABSTRACT
Celiac disease (CD)also known as gluten-sensitive enteropathyis a chronic, genetically predisposing and autoimmune entity with a wide range of clinical manifestations triggered by gluten ingestion, which affects 1% of the general population. Currently, up to 60% of the diagnosis of CD is in adults due to the atypical course of the disease. The severe acute onset of CDalso called celiac crisisis very uncommon and is still not well documented in adults. We report the case of a 58-year-old man who presented a 45-day history of subtle-onset diarrhea followed by malabsorption syndrome with progressive weight loss, anasarca, and electrolyte disturbances. The diagnostic work-up included an upper digestive endoscopy, which showed scalloping of the duodenal mucosa with pathological features confirmed on biopsies. Specific antibodies were positive, and a satisfactory clinical response was obtained once a gluten-free diet was started. Celiac crisis is a rare initial presentation of CD characterized by severe diarrhea, dehydration, weight loss, hypoproteinemia, and metabolic and electrolyte disturbances. Although rare, it should be considered in patients with apparently unexplained chronic diarrhea.
Subject(s)
Humans , Male , Middle Aged , Celiac Disease/diagnosis , Diarrhea/etiology , Malabsorption Syndromes/etiology , Celiac Disease/pathology , Diet, Gluten-Free , Gliadin/therapeutic use , Transglutaminases/therapeutic useABSTRACT
Apresentamos um caso de infecção do sistema nervoso central (SNC) por Penicillium spp em paciente do sexo masculino, HIV-negativo no Brasil. O paciente apresentou-se ao Serviço de Urgência do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo queixando-se de alteração visual e dificuldade na fala. Exames de neuroimagem mostraram lesões múltiplas, compatíveis com abscessos. A biópsia esterotáxica revelou infecção fúngica, iniciando-se o tratamento com anfotericina B com sucesso inicial. O paciente morreu poucos dias depois, vítima de uma hemorragia digestiva maciça devido a varizes de esôfago. A necropsia e a análise microbiológica final da biópsia cerebral revelaram infecção por Penicillium spp. Exixtem centenas de espécies de fungos do gênero Penicillium. A peniciliose sistêmica é causada pelo P. marneffei e costumava ser uma doença rara, mas atualmente é uma das infecções oportunistas mais comuns em associação com AIDS no Sudeste Asiático. Infecção pelo Penicillium spp de espécie diferente do P. marneffei normalmente causa apenas doenças superficiais ou alérgicas mas doenças invasivas também ocorrem raramente. Nós relatamos o quarto caso de infecção do SNC por Penicillium spp.