RITUXIMAB NO TRATAMENTO DE PÊNFIGO VULGAR REFRACTÁRIO

Pemphigus vulgaris is a rare autoimmune blistering disease affecting the skin and mucous membranes. In general, it has a severe course and may require long-term treatment with systemic corticosteroids and other immuno- suppressive drugs, which can lead to serious adverse events. Rituximab is a chimeric monoclonal antibody that targets the CD20 antigen found on B cells. Recently, there have been some reports documenting its use in the treatment of re- fractory pemphigus. The authors describe two clinical cases of pemphigus vulgaris refractory to conventional therapies that have been treated with rituximab and have achieved complete remission. In conclusion, our experience shows that rituximab is a valuable and safe option to treat severe and refractory pemphigus vulgaris KEYWORDS – Antibodies, monoclonal; Pemphigus. O pênfigo vulgar é uma doença bolhosa auto-imune rara, que atinge a pele e as mucosas. Geralmente tem um curso clínico severo, sendo necessário o recurso a terapêutica prolongada com corticóides sistémicos e outros fármacos imunossupressores, que podem conduzir a efeitos adversos graves. O rituximab é um anticorpo monoclo- nal quimérico dirigido ao antigénio CD20, expresso pelos linfócitos B. Recentemente, têm surgido alguns estudos que documentam o seu sucesso terapêutico no tratamento de pênfigo refractário. Os autores descrevem dois casos clínicos de pênfigo vulgar refractários às terapêuticas convencionais, que foram tratados com rituximab, tendo atin- gido a remissão completa da doença. A experiência bem sucedida em relação a estes dois casos clínicos reforça que o rituximab constitui uma opção valiosa e segura na abordagem terapêutica do pênfigo vulgar severo e refractário. PALAVRAS-CHAVE – Pênfigo vulgar; Rituximab.

Clonal chromosomal mosaicism and loss of chromosome Y in elderly men increase vulnerability for SARS-CoV-2

The pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2, COVID-19) had an estimated overall case fatality ratio of 1.38% (pre-vaccination), being 53% higher in males and increasing exponentially with age. Among 9578 individuals diagnosed with COVID-19 in the SCOURGE study, we found 133 cases (1.42%) with detectable clonal mosaicism for chromosome alterations (mCA) and 226 males (5.08%) with acquired loss of chromosome Y (LOY). Individuals with clonal mosaic events (mCA and/or LOY) showed a 54% increase in the risk of COVID-19 lethality. LOY is associated with transcriptomic biomarkers of immune dysfunction, pro-coagulation activity and cardiovascular risk. Interferon-induced genes involved in the initial immune response to SARS-CoV-2 are also down-regulated in LOY. Thus, mCA and LOY underlie at least part of the sex-biased severity and mortality of COVID-19 in aging patients. Given its potential therapeutic and prognostic relevance, evaluation of clonal mosaicism should be implemented as biomarker of COVID-19 severity in elderly people. Among 9578 individuals diagnosed with COVID-19 in the SCOURGE study, individuals with clonal mosaic events (clonal mosaicism for chromosome alterations and/or loss of chromosome Y) showed an increased risk of COVID-19 lethality

Case for diagnosis Caso para diagnóstico

We report a clinical case of a rare variant of pemphigus - pemphigus herpetiformis - which combines the clinical features of dermatitis herpetiformis with the immunological findings of pemphigus. Due to its atypical presentation, it is frequently misdiagnosed as dermatitis herpetiformis. It is basically characterized by the herpetiform pattern of skin lesions, severe pruritus and by the presence of eosinophilic spongiosis confirmed on histopathology. We call attention to the excellent response to dapsone.Descrevemos um caso clínico de uma variante rara de pênfigo - pênfigo herpetiforme - que combina os aspectos clínicos da dermatite herpetiforme com os achados imunológicos do pênfigo. Devido à sua apresentação atípica, é frequentemente diagnosticado equivocamente como dermatite herpetiforme. Caracteriza-se essencialmente pelo padrão herpetiforme das lesões cutâneas, prurido intenso e presença de espongiose eosinofílica no exame histopatológico. Enfatizamos a excelente resposta terapêutica à dapsona

Primary cutaneous adenoid cystic carcinoma of the abdomen: a rare entity

Adenoid cystic carcinoma is a rare neoplasm that arises from secretory glands, most frequently from the salivary glands. Primary cutaneous adenoid cystic carcinoma is microscopically identical to adenoid cystic carcinoma developing at other tissues. Therefore, differentiating between a primary cutaneous adenoid cystic carcinoma and an extracutaneous adenoid cystic carcinoma with cutaneous metastases is pivotal to determine its prognosis and management. We describe a case of primary cutaneous adenoid cystic carcinoma on the abdomen that was successfully treated with wide excision

Primary cutaneous adenoid cystic carcinoma of the abdomen: a rare entity

Adenoid cystic carcinoma is a rare neoplasm that arises from secretory glands, most frequently from the salivary glands. Primary cutaneous adenoid cystic carcinoma is microscopically identical to adenoid cystic carcinoma developing at other tissues. Therefore, differentiating between a primary cutaneous adenoid cystic carcinoma and an extracutaneous adenoid cystic carcinoma with cutaneous metastases is pivotal to determine its prognosis and management. We describe a case of primary cutaneous adenoid cystic carcinoma on the abdomen that was successfully treated with wide excisioninfo:eu-repo/semantics/publishedVersio

Adult blaschkolinear acquired inflammatory skin eruption (BLAISE) with simultaneous features of lichen striatus and blaschkitis

Blaschkitis and lichen striatus are generally distinguished in the literature by the age of onset, lesion distribution, and histopathology. However, there is currently no clear consensus among authors about whether to consider blaschkitis and lichen striatus different clinical entities or a spectrum ofthe same disease. We present a case of adult BLAISE with features of both lichen striatus and blaschkitis, which seems to support the theory that these clinical entities may in fact represent a spectrum of the same pathological process