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1.
Medicina (B Aires) ; 84(5): 1007-1010, 2024.
Artículo en Español | MEDLINE | ID: mdl-39399945

RESUMEN

The relationship between bullous pemphigoid (BP) and neoplasms has been debated in the medical literature. Although numerous case reports have described the coexistence of BP with various neoplasms, case-control studies have yielded conflicting results. We present the case of a male patient who developed BP shortly after being diagnosed with mycosis fungoides (MF). He was a 77-year-old man with a history of type 2 diabetes mellitus and hypertension who was diagnosed with MF. Subsequently, he developed blisters after sun exposure, and was diagnosed with BP through histopathology and direct immunofluorescence. The patient was treated with prednisone and methotrexate, with favorable evolution without recurrence of MF or appearance of new blisters. The association between cutaneous T-cell lymphoma and autoimmune blistering disease is rare, although similar cases have been reported, some associated with phototherapy. In this case, the onset of BP after sun exposure suggests a potential connection. The coexistence of BP and MF remains controversial, and this case highlights the importance of considering autoimmune blistering diseases in patients with oncohematological neoplasms.


La relación entre el penfigoide ampollar (PA) y las neoplasias ha sido objeto de debate en la literatura médica. Aunque numerosos informes de casos han descrito la coexistencia del PA con diversas neoplasias, estudios de casos y controles han arrojado resultados contradictorios. Presentamos el caso de un paciente masculino que desarrolló un PA poco después de ser diagnosticado con una micosis fungoide (MF). Se trata de un hombre de 77 años con antecedentes de diabetes mellitus tipo 2 e hipertensión arterial que fue diagnosticado con MF. Posteriormente, desarrolló ampollas después de una exposición solar, siendo diagnosticado con PA mediante histopatología e inmunofluorescencia directa. El paciente fue tratado con meprednisona y metotrexato, evolucionando favorablemente sin recurrencia de MF ni aparición de nuevas ampollas. La asociación entre un linfoma cutáneo de células T y una enfermedad ampollar autoinmune es rara, aunque han sido reportados casos similares, algunos asociados con fototerapia. En este caso la aparición del PA después de la exposición solar sugiere una conexión potencial. La coexistencia entre PA y MF sigue siendo controvertida, y este caso destaca la importancia de considerar enfermedades ampollares autoinmunes en pacientes con neoplasias oncohematológicas.


Asunto(s)
Micosis Fungoide , Penfigoide Ampolloso , Neoplasias Cutáneas , Humanos , Penfigoide Ampolloso/complicaciones , Penfigoide Ampolloso/diagnóstico , Penfigoide Ampolloso/etiología , Micosis Fungoide/complicaciones , Micosis Fungoide/patología , Micosis Fungoide/diagnóstico , Masculino , Anciano , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/patología , Prednisona/uso terapéutico , Metotrexato/uso terapéutico
2.
Front Immunol ; 15: 1419054, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39318633

RESUMEN

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease in humans, characterized by tense blisters, erosions, urticarial lesions, and itching on normal or erythematous skin. Many autoimmune diseases are considered comorbidities of BP, but clinical case reports of BP complicated by Sjögren's syndrome are very scarce. Furthermore, cases of central nervous system infection secondary to both autoimmune diseases are even rarer. Case presentation: We report a 74-year-old woman diagnosed with bullous pemphigoid, who showed relief of active lesions after treatment with methylprednisolone and dupilumab injections. However, she was admitted for pulmonary infection during which she was diagnosed with Sjögren's syndrome (SS). Subsequently, the patient developed altered consciousness, indicating a central nervous system infection. Adjustment of steroid dosage and aggressive antimicrobial therapy led to alleviation of symptoms. Conclusion: The coexistence of autoimmune subepidermal blistering diseases and SS is rare. The role of SS in the pathogenesis of skin lesions is unclear, and the relationship between these blistering diseases and SS remains elusive. Further research is needed to determine whether there are common pathological mechanisms between the two conditions.


Asunto(s)
Infecciones del Sistema Nervioso Central , Penfigoide Ampolloso , Síndrome de Sjögren , Humanos , Femenino , Penfigoide Ampolloso/tratamiento farmacológico , Penfigoide Ampolloso/diagnóstico , Penfigoide Ampolloso/complicaciones , Penfigoide Ampolloso/etiología , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Síndrome de Sjögren/tratamiento farmacológico , Síndrome de Sjögren/inmunología , Anciano , Infecciones del Sistema Nervioso Central/complicaciones , Infecciones del Sistema Nervioso Central/tratamiento farmacológico , Infecciones del Sistema Nervioso Central/diagnóstico , Metilprednisolona/uso terapéutico , Anticuerpos Monoclonales Humanizados/uso terapéutico
6.
J Am Acad Dermatol ; 91(1): 82-90, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38492858

RESUMEN

BACKGROUND: Autoimmune blistering disorders (ABDs) might elevate cardiovascular risk, but studies are lacking. OBJECTIVE: The objective of this study was to examine if ABDs elevate the risk of atherosclerotic cardiovascular disease, heart failure, arrhythmia, venous thromboembolism, and cardiovascular death. METHODS: A population-based cohort of Danish patients with ABD (≥18 years of age) diagnosed during 1996-2021 (n = 3322) was compared with an age- and sex-matched comparison cohort from the general population (n = 33,195). RESULTS: Compared with the general population, patients with ABDs had higher 1-year risks of atherosclerotic cardiovascular disease (3.4% vs 1.6%), heart failure (1.9% vs 0.7%), arrhythmia (3.8% vs 1.3%), venous thromboembolism (1.9% vs 0.3%), and cardiovascular death (3.3% vs 0.9%). The elevated risk persisted after 10 years for all outcomes but arrhythmia. The hazard ratios associating ABDs with the outcomes during the entire follow-up were 1.24 (1.09-1.40) for atherosclerotic cardiovascular disease, 1.48 (1.24-1.77) for heart failure, 1.16 (1.02-1.32) for arrhythmia, 1.87 (1.50-2.34) for venous thromboembolism, and 2.01 (1.76-2.29) for cardiovascular death. The elevated cardiovascular risk was observed for both pemphigus and pemphigoid. LIMITATIONS: Our findings might only generalize to patients with ABDs without prevalent cardiovascular diseases. CONCLUSION: Patients with ABDs had an elevated cardiovascular risk compared with age- and sex-matched controls.


Asunto(s)
Enfermedades Autoinmunes , Enfermedades Cardiovasculares , Humanos , Masculino , Femenino , Persona de Mediana Edad , Dinamarca/epidemiología , Anciano , Adulto , Enfermedades Autoinmunes/epidemiología , Enfermedades Autoinmunes/complicaciones , Enfermedades Cardiovasculares/epidemiología , Enfermedades Cardiovasculares/mortalidad , Tromboembolia Venosa/epidemiología , Tromboembolia Venosa/etiología , Estudios de Cohortes , Insuficiencia Cardíaca/epidemiología , Pénfigo/epidemiología , Pénfigo/complicaciones , Medición de Riesgo/estadística & datos numéricos , Estudios de Casos y Controles , Enfermedades Cutáneas Vesiculoampollosas/epidemiología , Aterosclerosis/epidemiología , Arritmias Cardíacas/epidemiología , Anciano de 80 o más Años , Penfigoide Ampolloso/epidemiología , Penfigoide Ampolloso/complicaciones , Factores de Riesgo de Enfermedad Cardiaca , Adulto Joven
7.
Int J Dermatol ; 63(5): 572-579, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38217028

RESUMEN

We aimed to systematically review and meta-analyze the association between diabetes mellitus (DM) and bullous pemphigoid (BP). Bullous pemphigoid (BP) is a prevalent autoimmune subepidermal blistering disease. Comorbid health conditions like neurological diseases and malignancies have been associated with BP. Growing evidence suggests that type 2 diabetes mellitus (T2DM) may increase the risk of developing BP. This review aims to synthesize this evidence. A systematic literature review was performed using Medline, PubMed, and Scopus in March 2022. Studies exploring the association between BP and DM were included. Data were extracted, and quality was assessed using the Newcastle-Ottawa scale. Meta-analysis was conducted to identify the odds ratio (OR) and 95% confidence intervals (CI) of the association. Seventeen studies were included, most being case-control studies from Europe and Asia. The pooled OR was 2.06 (95% CI: 1.61-2.62), suggesting a significant association between DM and BP. However, strong heterogeneity (I2 = 88%) was observed. Evidence consolidates a significant relationship between DM and BP, potentially due to alterations in the immune system and skin properties caused by diabetes. Strengths of this review include a comprehensive search, rigorous methodology, large sample size, and heterogeneity evaluation. However, varying study quality, potential publication bias, and unaccounted confounding factors present limitations. There is a potential link between T2DM and an increased risk of BP. Further studies are required to understand this association and the underlying mechanisms.


Asunto(s)
Diabetes Mellitus Tipo 2 , Penfigoide Ampolloso , Humanos , Comorbilidad , Diabetes Mellitus Tipo 2/epidemiología , Diabetes Mellitus Tipo 2/complicaciones , Penfigoide Ampolloso/epidemiología , Penfigoide Ampolloso/complicaciones , Penfigoide Ampolloso/inmunología , Factores de Riesgo
8.
J Dermatol ; 51(3): 403-408, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38212903

RESUMEN

Bullous pemphigoid (BP) is the most common autoimmune blistering disease, which primarily affects the elderly. However, the relationship between BP and malignancy remains controversial in traditional observational studies. The aim of this study, which included only European populations, was to assess the potential causative link between BP and 13 types of malignant tumors in a two-sample Mendelian randomization (MR) study. BP was not associated with an increased risk of developing 13 types of malignant tumors. This study did not find a causal relationship between BP and malignant tumors. However, further research is warranted to examine the generalizability of this conclusion in non-European populations.


Asunto(s)
Enfermedades Autoinmunes , Neoplasias , Penfigoide Ampolloso , Humanos , Anciano , Penfigoide Ampolloso/epidemiología , Penfigoide Ampolloso/genética , Penfigoide Ampolloso/complicaciones , Análisis de la Aleatorización Mendeliana , Neoplasias/epidemiología , Neoplasias/genética , Neoplasias/complicaciones , Vesícula , Enfermedades Autoinmunes/complicaciones
10.
Ocul Immunol Inflamm ; 32(1): 62-70, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-36637982

RESUMEN

PURPOSE: To evaluate ocular surface involvement, tear cytokine levels, and histopathological changes in pemphigus and pemphigoid patients. METHODS: A total of 22 patients (15 pemphigus and 7 pemphigoids) and 21 non-diseased controls were enrolled in our study. All participants underwent ocular surface evaluation, which included ocular surface disease index test, slit lamp observation, dry eye-related examination, tear multicytokine analysis, and conjunctival impression cytology. RESULTS: Pemphigus and pemphigoid patients presented much more severe conjunctivochalasis, corneal epithelial defects, corneal opacity, symblepharon   and dry eye. Severe ocular surface squamous metaplasia and a significant increase of tear macrophage inflammatory protein-1beta, tumor necrosis factor-alpha, interleukin (IL)-1ß, IL -6, and IL-8 occurred in pemphigus and pemphigoid patients. CONCLUSIONS: Our results revealed that ocular surface inflammation and dry eye persist in most pemphigus and pemphigoid patients, and do not occur in parallel with the systemic course. Regular ophthalmological examinations and local anti-inflammatory should be provided for pemphigus and pemphigoid patients.


Asunto(s)
Enfermedades de la Conjuntiva , Síndromes de Ojo Seco , Penfigoide Ampolloso , Pénfigo , Humanos , Penfigoide Ampolloso/complicaciones , Penfigoide Ampolloso/diagnóstico , Pénfigo/complicaciones , Pénfigo/diagnóstico , Síndromes de Ojo Seco/diagnóstico , Síndromes de Ojo Seco/etiología , Síndromes de Ojo Seco/patología , Enfermedades de la Conjuntiva/diagnóstico , Enfermedades de la Conjuntiva/etiología , Conjuntiva/patología , Lágrimas , Interleucina-1beta , Inflamación/diagnóstico , Inflamación/patología
15.
Int Immunopharmacol ; 125(Pt A): 111082, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37913571

RESUMEN

BACKGROUND: Previous studies have revealed the factors associated with the relapse of bullous pemphigoid (BP). This study aims to assess the characteristics of BP patients at the time of initial diagnosis and a potential association with subsequent relapse occurrences. METHODS: A retrospective cohort study was conducted on 205 BP patients from January 2009 to May 2022 at the Peking Union Medical College Hospital in Beijing, China. The median follow-up duration was 2.7 years. We conducted univariate and multivariate analyses on various clinical indicators (e.g., lesional involvements and medical history) and laboratory test results. RESULTS: Among the 205 patients, 118 (57.6 %) relapsed during the follow-up period. Univariate analysis revealed several factors associated with relapse, which were greater age [Hazard Ratio (HR)1.018, 95 % confidence interval (CI) 1.004-1.032] (p = 0.010), a past medical history of thyroid diseases [HR 3.674, 95 % CI 1.472-9.167] (p = 0.005), hematological disease complications [HR 4.123, 95 % CI 1.301-13.061] (p = 0.016), negative C3 deposition in direct immunofluorescence [HR 0.574, 95 % CI 0.374-0.883] (p = 0.011) and prealbumin level less than 200 mg/L[HR 0.580, 95 % CI 0.351-0.957] (p = 0.033). Multivariate analysis demonstrated that patients with negative C3 deposition in direct immunofluorescence [HR 0.524, 95 % CI 0.296-0.927] (p = 0.026) and prealbumin levels below 200 mg/L [HR 0.541, 95 % CI 0.301-0.974] (p = 0.041) were associated with further relapses of BP. CONCLUSIONS: Negative C3 deposition in direct immunofluorescence and a prealbumin level below 200 mg/L at initial diagnosis served as predictive markers for future relapses of BP. Systemic evaluation of BP patients at initial diagnosis could be essential in helping prevent recurrences and achieve more effective disease management.


Asunto(s)
Penfigoide Ampolloso , Humanos , Penfigoide Ampolloso/diagnóstico , Penfigoide Ampolloso/complicaciones , Prealbúmina , Estudios Retrospectivos , Factores de Riesgo , Enfermedad Crónica , Recurrencia
16.
Ocul Surf ; 30: 142-149, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37690517

RESUMEN

PURPOSE: Ocular Mucous Membrane Pemphigoid (OcMMP) is an orphan disease characterized by chronic autoimmune-driven conjunctival inflammation leading to progressive scarring, debilitating symptoms, and blinding sequelae. This feasibility study aims to demonstrate conjunctival genetic transcriptomic analyses as a putative tool for interrogation of pathogenic signaling pathways in OcMMP. METHODS: Conjunctival RNA profiling using the NanoString nCounter Human Fibrosis panel was undertaken on RNA extracted from conjunctival swabs obtained from 6 MMP patients (8 eyes; 4 M/2F; median age 78 [range 64-84] years); and 8 age-matched control participants (15 eyes; 3 M/5F; median age 69.5 [range 69-88] years). Data from 770 genes were analyzed with ROSALIND HyperScale architecture and stratified according to the level of clinically visible bulbar conjunctival inflammation. Normalization, fold-changes (≥+1.5-fold or ≤ -1.5-fold) and p-values adjustment (<0.05) using the Benjamini-Hochberg method were calculated. RESULTS: 93 differentially expressed genes (DEGs) were observed between OcMMP versus controls of which 48 were upregulated, and 45 downregulated. The top 4 upregulated DEGs represented fibrosis (COL3A1, COL1A1, FN1 and THBS1) while the key under-expressed genes (SCIN, HMGS2, XCL1/2) were indicative of ocular surface failure (goblet cell loss, keratinization, vulnerability to secondary infections). Forty-four pathways had a global significance score ≥2, the most significant being those related to extracellular matrix (ECM) remodeling, synthesis, and degradation. These pathways were accentuated in eyes with visible inflammation. CONCLUSIONS: NanoString methodology acquired via a simple conjunctival swab identifies profibrotic genes in OcMMP group and differentiates inflamed eyes. Longitudinal sampling and following investigative intervention will further mechanistic insight and development of novel biomarkers to monitor disease progression.


Asunto(s)
Enfermedades de la Conjuntiva , Conjuntivitis , Penfigoide Benigno de la Membrana Mucosa , Penfigoide Ampolloso , Humanos , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Penfigoide Ampolloso/complicaciones , Penfigoide Ampolloso/metabolismo , Penfigoide Ampolloso/patología , Conjuntiva/patología , Penfigoide Benigno de la Membrana Mucosa/genética , Fibrosis , Inflamación/metabolismo , Membrana Mucosa , Perfilación de la Expresión Génica , ARN/metabolismo , Enfermedades de la Conjuntiva/metabolismo
18.
Immun Inflamm Dis ; 11(7): e924, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-37506153

RESUMEN

OBJECTIVE: To retrospectively analyze the efficacy and safety of dupilumab in the treatment of bullous pemphigoid. METHODS: From October 2020 to October 2022, the medical records of patients with bullous pemphigoid who were treated with dupilumab in our department were collected retrospectively to analyze the therapeutic effect and changes in laboratory indexes. RESULTS: The records of a total of 11 patients with bullous pemphigoid who were treated with dupilumab was reviewed. Within 2 weeks of the treatment, 10 (90.9%) of the 11 patients had complete or substantial control of the disease. The BPDAI scores of the patients decreased from baseline 113 (62, 181) to 37 (6, 130) at 2 weeks (p = .001) and 4 (0, 37) at 12 weeks after treatment (p < .001). In the 11 patients treated with dupilumab, the relief time of pruritus was 0-3 days (0.5, 7) days, and the pruritus was significantly alleviated after 2 weeks (t = 15.925, p < .001). The DLQI score decreased from (25.5 ± 2.5) before treatment, to (11.8 ± 4.4) at 2 weeks (t = 10.764, p < .001) and (2.1 ± 1.9) at 12 weeks (t = 30.038, p < .001). The patients had high eosinophil counts, high serum IgE levels, low serum total protein levels, and abnormal blood coagulation function. The aforementioned indicators gradually returned to normal after treatment. No adverse reactions occurred during the treatment. CONCLUSION: Dupilumab can effectively control the condition of bullous pemphigoid, efficiently relieve pruritus symptoms, and is relatively safe.


Asunto(s)
Penfigoide Ampolloso , Humanos , Penfigoide Ampolloso/tratamiento farmacológico , Penfigoide Ampolloso/complicaciones , Penfigoide Ampolloso/diagnóstico , Glucocorticoides , Estudios Retrospectivos , Prurito/tratamiento farmacológico , Prurito/etiología , Gravedad del Paciente
20.
JAMA Dermatol ; 159(7): 750-756, 2023 07 01.
Artículo en Inglés | MEDLINE | ID: mdl-37285147

RESUMEN

Importance: Studies have linked bullous pemphigoid (BP) with venous thromboembolism (VTE) across several data sources finding 6-fold to 15-fold increased incidence rates. Objective: To determine the incidence of VTE in patients with BP compared with similar controls. Design, Setting, and Participants: This cohort study used insurance claims data from a nationwide US health care database from January 1, 2004, through January 1, 2020. Patients with dermatologist-recorded BP were identified (≥2 diagnoses of BP [International Classification of Diseases, Ninth Revision (ICD-9) 694.5 and ICD-10 L12.0] recorded by dermatologists within 1 year). Risk-set sampling identified comparator patients without BP and free of other chronic inflammatory skin diseases. Patients were followed-up until the first of the following events occurred: VTE, death, disenrollment, or end of data stream. Exposures: Patients with BP compared with those without BP and free of other chronic inflammatory skin diseases (CISD). Main outcome: Venous thromboembolism events were identified and incidence rates were computed before and after propensity-score (PS) matching to account for VTE risk factors. Hazard ratios (HRs) compared the incidence of VTE in BP vs non-CISD. Results: Overall, 2654 patients with BP and 26 814 comparator patients without BP or another CISD were identified. The mean (SD) age in the BP group was 73.0 (12.6) years and 55.0 (18.9) years in the non-CSID group. With a median follow-up time was 2 years, the unadjusted incidence rate (per 1000 person-years) of outpatient or inpatient VTE was 8.5 in the BP group compared with 1.8 in patients without a CISD. Adjusted rates were 6.7 in the BP group compared with 3.0 in the non-CISD group. Age-specific adjusted incidence rates (per 1000 person-years) in patients aged 50 to 74 years was 6.0 (vs 2.9 in the non-CISD group) and in those aged 75 years or older was 7.1 (vs 4.53 in the non-CISD group). After 1:1 propensity-score matching including 60 VTE risk factors and severity markers, BP was associated with a 2-fold increased risk of VTE (2.24 [1.26-3.98]) vs those in the non-CISD group. When restricting to patients aged 50 years or older, the adjusted relative risk of VTE was 1.82 (1.05-3.16) for the BP vs non-CISD groups. Conclusions: In this nationwide US cohort study, BP was associated with a 2-fold increased incidence of VTE after controlling for VTE risk factors in a dermatology patient population.


Asunto(s)
Penfigoide Ampolloso , Tromboembolia Venosa , Humanos , Tromboembolia Venosa/etiología , Tromboembolia Venosa/complicaciones , Estudios de Cohortes , Penfigoide Ampolloso/epidemiología , Penfigoide Ampolloso/complicaciones , Factores de Riesgo , Incidencia
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