RESUMEN
Psoriasis is a common cutaneous disease, and often these patients require treatment with biologics. Screening for latent tuberculosis is an important step in the pre-biologic work-up. A 58-year-old woman with moderate-to-severe psoriasis was found to be positive for latent tuberculosis during pre-biologic screening. She received rifampicin for 6 months and had complete resolution of her psoriasis, with persistent remission at 1-year follow-up. Improvement could be attributed to the immunomodulatory effects of rifampicin. Another theory proposes the existence of a tuberculosis-related type of psoriasis that improves when tuberculosis is adequately treated.
RESUMEN
Dupilumab is the only biologic therapy currently approved in Europe and the United States for severe atopic dermatitis in patients 6 years of age or older. Off-label use is rationalized in younger children with severe atopic dermatitis. Decisions about vaccination for children on dupilumab are complex and depend on both the child's current treatment and the type of vaccination required. To achieve consensus on recommendations for vaccination of pediatric patients with atopic dermatitis treated with or planning to start dupilumab, a review of the literature and a modified-Delphi process was conducted by a working group of 5 panelists with expertise in dermatology, immunology, infectious diseases and vaccination. Here, we provide seven recommendations for vaccination of pediatric patients with atopic dermatitis treated with or planning to start dupilumab. These recommendations serve to guide physicians' decisions about vaccination in children with atopic dermatitis treated with dupilumab. Furthermore, we highlight an unmet need for research to determine how significantly dupilumab affects cellular and humoral immune responses to vaccination with live attenuated and inactivated vaccines.
Asunto(s)
Anticuerpos Monoclonales Humanizados/efectos adversos , Conferencias de Consenso como Asunto , Dermatitis Atópica/tratamiento farmacológico , Guías de Práctica Clínica como Asunto , Vacunación/normas , Alergia e Inmunología/normas , Niño , Técnica Delphi , Dermatitis Atópica/diagnóstico , Dermatitis Atópica/inmunología , Dermatología/normas , Humanos , Inmunidad Celular/efectos de los fármacos , Inmunidad Humoral/efectos de los fármacos , Inmunogenicidad Vacunal , Índice de Severidad de la Enfermedad , Vacunación/efectos adversosRESUMEN
BACKGROUND: Although several therapeutic options have been suggested for alopecia areata (AA), none of them are consistently effective, thus making the management of severe or refractory cases challenging. Several studies have recently reported the usage of methotrexate (MTX) in AA; however, the pure effect of MTX monotherapy remains elusive. OBJECTIVE: To evaluate efficacy and safety of oral methotrexate monotherapy for AA. METHODS: We retrospectively reviewed the clinical course of AA patients including pediatric cases treated with MTX monotherapy. Their detailed clinical data including original severity of AA, final treatment outcome, the duration until the maximum response, and side effects, were assessed. Statistical analysis was performed to evaluate if the clinical factors including the duration of current alopecia, age, the presence of body hair loss, and sex were associated with treatment response. RESULTS: All included patients had severe AA and failed standard therapies. Thirteen out of 15 cases demonstrated improvement during the monotherapy, and all responders demonstrated the maximum response within 1 year. Female patients had significantly better outcomes than male patients. Other factors did not significantly influence on the treatment outcome. None of the patients experienced side effects that were severe enough to terminate the treatment. CONCLUSIONS: Our results support MTX monotherapy as a feasible option for severe AA patients who fail other standard therapies or for whom systemic corticosteroids are contraindicated.
Asunto(s)
Alopecia Areata/tratamiento farmacológico , Fármacos Dermatológicos/administración & dosificación , Metotrexato/administración & dosificación , Administración Oral , Adolescente , Adulto , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Adulto JovenAsunto(s)
Neoplasias de la Mama , Supervivientes de Cáncer , Mama , Femenino , Humanos , Piel , SobrevivientesRESUMEN
First described in 1996, the drug reaction, eosinophilia, and systemic symptoms syndrome (DReSS) is considered, along with Stevens-Johnson syndrome and toxic epidermal necrolysis, a severe cutaneous drug reaction. It is characterized by the presence of a maculopapular erythematous skin eruption, fever, lymphadenopathy, influenza-like symptoms, eosinophilia, and visceral involvement such as hepatitis, pneumonitis, myocarditis, pericarditis, nephritis, and colitis. The prognosis of patients with DReSS is related to the severity of visceral involvement. The mortality ranges from approximately 5% to 10%, and death is mainly due to liver failure, which is also the organ most commonly involved in this syndrome. Although it was previously hypothesized in 1994, DReSS syndrome can lead to reactivation of one or more human herpesvirus family members. Now being included as diagnostic criteria in a proposed diagnostic score system, this reactivation can be detected up to 2-3 wk after DReSS syndrome onset. Other causes of mortality in DReSS syndrome include myocardial or pulmonary lesions and hemophagocytosis. We reviewed the literature of previously reported case-series of DReSS and liver involvement, highlighting the pattern of liver damage, the treatment used, and the outcome.
RESUMEN
There are many autoimmune diseases associated with primary biliary cholangitis (PBC), known as primary biliary cirrhosis; however, the association between PBC and warm autoimmune hemolytic anemia (wAIHA) has rarely been reported. It is documented that hemolysis is present in over 50% of the patients with chronic liver disease, regardless of the etiologies. Due to the clear and frequent relationship between PBC and many autoimmune diseases, it is reasonable to suppose that wAIHA may be another autoimmune disorder seen in association with PBC. Here we reported a 53-year-old female patient diagnosed with wAIHA associated with PBC.
Asunto(s)
Anemia Hemolítica Autoinmune/complicaciones , Cirrosis Hepática Biliar/complicaciones , Anemia Hemolítica Autoinmune/tratamiento farmacológico , Biopsia , Quimioterapia Combinada , Femenino , Glucocorticoides/uso terapéutico , Humanos , Hígado/patología , Cirrosis Hepática Biliar/diagnóstico , Cirrosis Hepática Biliar/tratamiento farmacológico , Cirrosis Hepática Biliar/patología , Persona de Mediana Edad , Prednisolona/uso terapéutico , Ácido Ursodesoxicólico/uso terapéuticoAsunto(s)
Adenocarcinoma/complicaciones , Adenocarcinoma/secundario , Neoplasias Colorrectales/complicaciones , Neoplasias Colorrectales/patología , Elefantiasis/etiología , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/diagnóstico , Adenocarcinoma/diagnóstico , Adulto , Elefantiasis/diagnóstico , Femenino , Humanos , Linfedema/complicaciones , Linfedema/patología , Neoplasias Cutáneas/secundarioRESUMEN
Cutaneous amyloidosis is a rare disease characterized by the deposition of amyloid in the dermis. It can be primary or secondary, depending on associated diseases. It has been linked to various autoimmune diseases, including primary biliary cirrhosis. We present the case of a patient with an autoimmune hepatitis-primary biliary cirrhosis overlap syndrome with concomitant cutaneous amyloidosis, a very unusual association, and discuss similar cases and possible pathophysiological implications.
Asunto(s)
Amiloidosis Familiar/etiología , Autoinmunidad , Hepatitis Autoinmune/complicaciones , Cirrosis Hepática Biliar/complicaciones , Enfermedades Cutáneas Genéticas/etiología , Adulto , Amiloidosis Familiar/diagnóstico , Amiloidosis Familiar/inmunología , Biopsia , Diagnóstico Diferencial , Hepatitis Autoinmune/diagnóstico , Hepatitis Autoinmune/inmunología , Humanos , Hígado/patología , Cirrosis Hepática Biliar/diagnóstico , Cirrosis Hepática Biliar/inmunología , Masculino , Piel/patología , Enfermedades Cutáneas Genéticas/diagnóstico , Enfermedades Cutáneas Genéticas/inmunología , SíndromeAsunto(s)
Vasculitis Leucocitoclástica Cutánea/diagnóstico , Diagnóstico Diferencial , Dermatosis de la Mano/diagnóstico , Dermatosis de la Mano/patología , Humanos , Masculino , Piel/patología , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/patología , Vasculitis Leucocitoclástica Cutánea/patologíaRESUMEN
BACKGROUND: The prevalence and features of graft-versus-host disease (GVHD) in patients receiving allografts using peripheral blood stem cells (PBSCs) after a reduced-intensity conditioning (RIC) regimen are not well known. Several features of GVHD in patients at two institutions using RIC were assessed. METHODS: We analysed the overall survival (OS) and prevalence of GVHD in patients who underwent outpatient allogeneic PBSC transplantation after RIC between October 1998 and July 2008. RESULTS: We included 301 patients with a median age of 30 yrs (range, 1-71 yrs). In 37 cases, allogeneic peripheral blood stem cell transplantation was indicated for non-malignant disease, and in 264 for malignant disease. The median OS was 35 months. The estimated 3-yr OS was 48%. A total of 154 patients developed GVHD: there were 64 acute, 50 chronic and 40 cases that progressed from acute to chronic. Of the 104 patients with acute GVHD (aGVHD), 40% had grade I and 60% had grades II-IV. Of the 90 patients with chronic GVHD (cGVHD), 67% had limited and 33% had extensive forms. A total of 160 patients died, 40 as a result of GVHD (24 from aGVHD and 16 from cGVHD), 50 as a result of progressive disease and 70 from diverse causes. CONCLUSIONS: The incidence of GVHD was lower than in other series using conventional myeloablative preparative regimens. Most importantly, the severity of GVHD did not significantly affect the long-term survival.