RESUMO
Rituximab (RTX) is a monoclonal antibody directed against CD20 antigen indicated in an increasing number of immune-mediated diseases. While its efficacy in pemphigus vulgaris has been widely investigated, only a few data about its possible role in pemphigoid diseases have been reported in the literature. Accordingly, herein we evaluated a case series of patients with mucous membrane pemphigoid (MMP) treated with RTX. We included patients with a history of severe/refractory MMP who received at least one cycle of intravenous RTX between May 2018 and December 2021 and had 6 months of follow-up time. Disease control (DC) was our early endpoint, while complete remission (CR) and partial remission (PR) were late endpoints. CR off-therapy, relapses, and adverse events were evaluated as well. Our population included 10 MMP patients. Eight out of ten patients (80%) achieved DC in a mean of 8 weeks, while two patients with ocular MMP were non-responders. Among the eight patients who achieved DC, two reached CR off therapy, two CR on minimal therapy, and two achieved PR on minimal therapy. In our case series, the addition of RTX to conventional therapies was demonstrated to be safe and effective in reaching rapid disease control in the majority of refractory MMP patients.
RESUMO
Bullous pemphigoid (BP) represents the most common autoimmune bullous disease and is characterized by IgG autoantibodies targeting collagen XVII (BP180). BP has reportedly been occurred in association with other inflammatory skin diseases. Here, we describe the unusual occurrence of BP in a female patient with a concomitant history of generalized morphea (localized scleroderma, LoS) and cutaneous and genital lichen sclerosus (LiS). The occurrence of BP was associated with elevated serum levels of anti-BP180 IgG autoantibodies, which decreased upon clinical remission. Autoimmune bullous diseases and sclerosing dermatitis are immunologically distinct entities, whose association has been rarely described. In this study, we provide a literature review on cases of BP developed in patients with either LoS or LiS. Further, we discussed immunological mechanisms which may have favored the emergence of BP in our patient.
Assuntos
Doenças Autoimunes , Líquen Escleroso e Atrófico , Penfigoide Bolhoso , Esclerodermia Localizada , Autoanticorpos , Doenças Autoimunes/complicações , Feminino , Humanos , Imunoglobulina G , Líquen Escleroso e Atrófico/complicações , Penfigoide Bolhoso/complicações , Esclerodermia Localizada/complicaçõesRESUMO
We describe an unusual case of myelodysplasia cutis in a patient with chronic myelomonocytic leukaemia.
Assuntos
Leucemia Mielomonocítica Crônica , Leucemia Mielomonocítica Juvenil , Síndromes Mielodisplásicas , Neoplasias Cutâneas , Humanos , Leucemia Mielomonocítica Crônica/complicações , Leucemia Mielomonocítica Crônica/diagnóstico , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/diagnóstico , Pele , Neoplasias Cutâneas/diagnósticoRESUMO
Vaccines are today considered one of the most effective means against the Sars-CoV-2 pandemic. The BNT162b2 vaccine by Pfizer/BioNTech has been massively administered throughout the globe; since its approval, a wide spectrum of cutaneous reactions has been reported. Here we report the case of a 52-year-old Caucasian male who presented with an acute febrile eruption that arose 72 h after the first dose of the BNT162b2 vaccine. The clinicopathological findings were consistent with Sweet's syndrome. The short latency time suggested a possible role of the vaccine in triggering Sweet's syndrome in this case.
Assuntos
Epidermólise Bolhosa Adquirida/diagnóstico , Imunoglobulina A/imunologia , Pele/patologia , Idoso , Biópsia , Colágeno Tipo VII/imunologia , Diagnóstico Diferencial , Epidermólise Bolhosa Adquirida/sangue , Epidermólise Bolhosa Adquirida/imunologia , Epidermólise Bolhosa Adquirida/patologia , Humanos , Imunoglobulina A/sangue , Masculino , Microscopia de Fluorescência , Pele/imunologiaRESUMO
Percutaneous central vascular venous access to allow positioning of a totally implantable access port (TIAP) is a widely employed procedure. An open issue is the need to find an ionizing radiation free technique which allows a safe and correct positioning of the device. Echo-guided vein puncture is currently accepted as the best method for decreasing major immediate complications. We describe an entirely echo-based method for the insertion and assessment of correct placement of the totally implantable access ports (TIAP). 20 TIAPs with the described technique have been placed. TIAP can be safely positioned by entirely echo-guided technique avoiding ionizing radiation imaging.