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1.
J Am Acad Dermatol ; 86(5): 1035-1041, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-34224771

RESUMEN

BACKGROUND: Cutaneous polyarteritis nodosa is a form of medium-sized vessel vasculitis. Despite a disabling and prolonged course, data on treatment efficacy and safety remain scarce. OBJECTIVES: We aimed to describe treatment efficacy and safety in patients with cutaneous polyarteritis nodosa. METHODS: This multicenter retrospective, observational study, recorded clinical and biologic data together with treatments received. The primary outcome was the rate of complete response at month 3. Secondary outcomes assessed drug survival and safety. RESULTS: We included 68 patients who received a median of 2 therapeutic lines (interquartile range, 1-3). Overall, complete response was achieved in 13 of 42 (31%) patients with colchicine, 4 of 17 (23%) with dapsone, 11 of 25 (44%) with glucocorticoids (GCs) alone, 1 of 9 (11%) with nonsteroidal anti-inflammatory drugs, 11 of 13 (84%) with GCs+azathioprine, and 7 of 15 (47%) with GCs+methotrexate. GCs+azathioprine had the best drug survival (median duration, 29.5 months; interquartile range, 19.5-36.0). Response at month 3 was decreased with peripheral neurologic involvement (odds ratio, 0.19; 95% confidence interval, 0.03-0.81; P = .04). Overall, the rate of treatment-related adverse events was 18%, which led to the discontinuation of treatment in 7% of patients. LIMITATION: Retrospective study. CONCLUSION: Colchicine seems to confer good benefit-risk balance in cutaneous polyarteritis nodosa without peripheral sensory neuropathy. GCs+azathioprine seem the best treatment in the event of relapse.


Asunto(s)
Poliarteritis Nudosa , Azatioprina/uso terapéutico , Colchicina/efectos adversos , Glucocorticoides/uso terapéutico , Humanos , Recurrencia Local de Neoplasia/tratamiento farmacológico , Poliarteritis Nudosa/tratamiento farmacológico , Estudios Retrospectivos
2.
Rheumatology (Oxford) ; 59(9): 2282-2286, 2020 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-31846040

RESUMEN

OBJECTIVES: Mucosal-associated invariant T (MAIT) cells are innate-like lymphocytes that are important for antibacterial immunity and may have regulatory roles. MAIT cells are decreased during SLE. However, their frequencies and phenotype have not been investigated in DM. We studied MAIT cell frequencies and phenotype in DM patients with active and inactive disease (after treatment). METHODS: Peripheral blood flow cytometry analysis of MAIT cells was compared between DM (n = 22), SLE (n = 10), psoriasis (n = 7) and atopic dermatitis (n = 5) patients, and healthy controls (n = 19). RESULTS: A dramatic decrease of circulating MAIT cell frequency was observed in active DM and SLE patients compared with healthy controls and other inflammatory skin diseases [active DM: median = 0.25% (interquartile range 0.19-0.6%), P < 0.0001; active SLE: median = 0.61 (0.55-0.77), P < 0.0001 vs healthy controls: 2.32% (1.18-4.45%)]. MAIT cells from active DM patients had an abnormal phenotype including increased expression of CD25 and cytotoxic T-lymphocyte-associated protein 4 that correlated with their low frequency in the blood. CONCLUSION: In DM, peripheral blood MAIT cells are dramatically reduced and have an activated/exhausted phenotype that may be linked to increased activation-induced cell death.


Asunto(s)
Dermatomiositis/sangre , Células T Invariantes Asociadas a Mucosa/metabolismo , Adulto , Dermatitis Atópica/sangre , Femenino , Citometría de Flujo , Humanos , Lupus Eritematoso Sistémico/sangre , Masculino , Persona de Mediana Edad , Fenotipo , Psoriasis/sangre , Índice de Severidad de la Enfermedad
3.
J Am Acad Dermatol ; 78(1): 107-114.e1, 2018 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-29061479

RESUMEN

BACKGROUND: Changing from one antimalarial (AM) agent to another is often recommended in cutaneous lupus erythematosus (CLE) when the first AM agent is ineffective or poorly tolerated. OBJECTIVE: To evaluate the effect on cutaneous response of a switch from hydroxychloroquine to chloroquine, or the reverse, after failure of the first AM agent. METHODS: We conducted a retrospective observational study between 1997 and September 2015. The overall cutaneous response rate and reasons for failure of the switch were assessed for up to 48 months. Kaplan-Meier survival curves were used to assess the risk for failure of the second AM agent. RESULTS: A total of 64 patients with CLE (78% were women) were included; for 48 patients, the switch was for inefficacy, and for 16, it was for adverse events. Median follow-up was 42 months (range, 3-171). Of the patients changed because of inefficacy, 56% were responders at month 3; however, the response decreased over time, with a median duration before failure of the second AM agent of 9 months (95% confidence interval, 6-24). For patients switched because of adverse events, the second AM agent was well tolerated in 69% of cases. LIMITATIONS: Retrospective design and subjective evaluation of cutaneous response. CONCLUSION: A change of AM agent should be considered in patients with CLE when the first AM agent is ineffective or poorly tolerated.


Asunto(s)
Antimaláricos/efectos adversos , Sustitución de Medicamentos , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/epidemiología , Lupus Eritematoso Cutáneo/tratamiento farmacológico , Insuficiencia del Tratamiento , Adulto , Anciano , Anciano de 80 o más Años , Antimaláricos/uso terapéutico , Cloroquina/efectos adversos , Cloroquina/uso terapéutico , Femenino , Estudios de Seguimiento , Francia , Hospitales Universitarios , Humanos , Hidroxicloroquina/efectos adversos , Hidroxicloroquina/uso terapéutico , Estimación de Kaplan-Meier , Lupus Eritematoso Cutáneo/diagnóstico , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Adulto Joven
8.
Acta Derm Venereol ; 97(7): 838-842, 2017 Jul 06.
Artículo en Inglés | MEDLINE | ID: mdl-28421232

RESUMEN

Langerhans cell histiocytosis is a rare histiocytic disorder for which skin involvement and management are poorly described in adults. The aim of this retrospective monocentric study in a national reference centre is to describe the clinical characteristics, quality of life, BRAF mutation status and outcomes of skin involvement in adult patients with Langerhans cell histiocytosis. Twenty-five patients (14 females, mean age 47 years) were included, with a median follow-up of 33 months (range 4-420 months). Patients experienced poor dermatological quality of life despite low body surface involvement. BRAFV600 mutations were detected in 8 of the 18 patients analysed (45%). Eight patients had an associated malignancy. Several treatment options were used and consisted of surgery, topical steroids and carmustine, thalidomide, methotrexate, vinblastine and steroids and cladribine. This study highlights the need to evaluate quality of life and to screen for associated malignancy in adult patients with Langerhans cell histiocytosis.


Asunto(s)
Histiocitosis de Células de Langerhans/genética , Histiocitosis de Células de Langerhans/terapia , Mutación , Proteínas Proto-Oncogénicas B-raf/genética , Calidad de Vida , Piel/patología , Adulto , Anciano , Anciano de 80 o más Años , Análisis Mutacional de ADN , Femenino , Francia , Marcadores Genéticos , Predisposición Genética a la Enfermedad , Histiocitosis de Células de Langerhans/enzimología , Histiocitosis de Células de Langerhans/patología , Humanos , Masculino , Persona de Mediana Edad , Fenotipo , Sistema de Registros , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento , Adulto Joven
12.
J Am Acad Dermatol ; 73(6): 1013-20, 2015 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-26464220

RESUMEN

BACKGROUND: Cutaneous polyarteritis nodosa (cPAN) is a skin medium vessel neutrophilic arteritis with livedo, nodules, and ulcerations. Macular lymphocytic arteritis (MLA) is a small arteritis with erythematous or pigmented macules and typical histologic features (a lymphocytic infiltrate, concentric fibrin ring, no disruption of the internal elastic lamina). OBJECTIVE: We sought to assess the frequency of clinical and histologic features of MLA in patients with cPAN. METHODS: This was a monocentric retrospective analysis of patients given the diagnosis of cPAN with blinded assessment of skin biopsy specimens. RESULTS: All 35 patients included had an infiltrated livedo, nodules, or both. Ulceration was rare. Erythematous or pigmented lesions were present in 54% of patients. Predominantly lymphocytic arteritis, a paucity of neutrophils, concentric fibrin ring, and absence of internal lamina elastic disruption were present in 60%, 20%, 18%, and 23% of patients, respectively. Median follow-up was 11 years. None of the patients had systemic involvement, and 57% had a complete remission. The incidence of complete remission was not different between patients having a predominant lymphocyte infiltrate or few neutrophils. LIMITATIONS: This was a retrospective, monocentric study without a control group of patients with MLA. CONCLUSIONS: Our data do not favor the classification of cPAN and MLA as distinct entities.


Asunto(s)
Arteritis/patología , Linfocitos/patología , Poliarteritis Nudosa/patología , Adulto , Distribución por Edad , Anciano , Anciano de 80 o más Años , Arteritis/diagnóstico , Arteritis/epidemiología , Biopsia con Aguja , Estudios de Cohortes , Bases de Datos Factuales , Diagnóstico Diferencial , Femenino , Francia , Humanos , Inmunohistoquímica , Incidencia , Estimación de Kaplan-Meier , Linfocitos/metabolismo , Masculino , Persona de Mediana Edad , Poliarteritis Nudosa/diagnóstico , Poliarteritis Nudosa/epidemiología , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Distribución por Sexo , Método Simple Ciego , Estadísticas no Paramétricas , Adulto Joven
14.
Blood ; 118(14): 3777-84, 2011 Oct 06.
Artículo en Inglés | MEDLINE | ID: mdl-21757618

RESUMEN

Xanthomas are a common manifestation of lipid metabolism disorders. They include hyperlipemic xanthoma, normolipemic xanthoma, and a related condition, necrobiotic xanthogranuloma (NXG). All 3 forms can be associated with monoclonal immunoglobulin (MIg). In an attempt to improve diagnosis, understanding, and treatment of this association, we retrospectively analyzed a personal series of 24 patients (2 hyperlipemic xanthoma, 11 normolipemic xanthoma, and 11 NXG) and 230 well-documented reports from the literature. With the exception of the nodules and plaques featured in NXG, the clinical presentation of xanthomatous lesions usually resembled that seen in common hyperlipidemic forms and could not be used to suspect MIg-associated xanthomas. Extracutaneous sites were not rare. The MIg was an IgG in 80% of cases. Myeloma was diagnosed in 35%. Hypocomplementemia with low C4 fraction was present in 80% of studied patients. Low C1 inhibitor serum levels were found in 53%. Cryoglobulinemia was detected in 27%. These abnormalities suggest immune complex formation because of interactions between the MIg and lipoproteins and argue in favor of a causal link between MIg and xanthomas. Monoclonal gammopathy therapy could thus be an option. Indeed, among the patients who received chemotherapy, hematologic remission was accompanied by improvement in xanthoma lesions in several cases.


Asunto(s)
Paraproteinemias/complicaciones , Paraproteinemias/terapia , Xantomatosis/complicaciones , Xantomatosis/terapia , Humanos , Paraproteinemias/diagnóstico , Paraproteinemias/patología , Estudios Retrospectivos , Piel/patología , Xantomatosis/diagnóstico , Xantomatosis/patología
17.
JAMA Dermatol ; 157(11): 1349-1354, 2021 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-34495287

RESUMEN

IMPORTANCE: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a recently described severe adult-onset autoinflammatory disease that is associated with myeloid lineage-restricted ubiquitin-activating enzyme 1 (UBA1) somatic variations that primarily affect the skin (Sweet syndrome), cartilage, and bone marrow. Skin symptoms have been poorly described. OBJECTIVE: To better describe clinical and pathological skin manifestations and their pathophysiology in VEXAS syndrome. DESIGN, SETTING, AND PARTICIPANTS: This multicenter retrospective case series study of clinical and histological features of 8 patients with VEXAS syndrome and skin involvement was conducted in France from December 2007 to March 2021, with molecular data obtained from March to April 2022. Any UBA1 variations were detected by Sanger or next-generation sequencing that was performed on bone marrow and formalin-fixed paraffin-embedded tissue sections of skin lesion biopsies. RESULTS: All 8 patients were men, and the median age at symptom onset was 65.5 years (interquartile range, 54-76 years). All patients had neutrophilic dermatosis skin lesions, including tender red or violaceous papules, sometimes edematous, without fever, arthralgia, recurrence or pathergy, inflammatory edematous papules on the neck and trunk (sometimes umbilicated), and firm erythematous purpuric or pigmented infiltrated plaques and nodules. Three patients had livedo racemosa. The infiltrates were perivascular and consisted of mature neutrophils with leukocytoclasia, which were admixed with myeloperoxidase-positive CD163-positive myeloid cells with indented nuclei and lymphoid cells in all cases. A sequencing analysis of paired bone marrow samples and skin lesion biopsies identified the same loss-of-function UBA1 variation in both samples for all patients. CONCLUSIONS AND RELEVANCE: This case series study describes the different clinical presentations of skin lesions found in VEXAS syndrome, which is characterized histologically by neutrophilic dermatosis. The findings suggested that the dermal infiltrates seen in VEXAS skin lesions are derived from the pathological myeloid clone. This suggests that using therapies that target the pathological clone may be effective in the long-term management of the disease.


Asunto(s)
Síndrome de Sweet , Enzimas Activadoras de Ubiquitina , Adulto , Médula Ósea , Humanos , Masculino , Mutación , Estudios Retrospectivos , Síndrome de Sweet/diagnóstico , Enzimas Activadoras de Ubiquitina/genética
19.
Arch Dermatol ; 143(8): 1046-50, 2007 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-17709664

RESUMEN

BACKGROUND: Schnitzler syndrome is characterized by chronic urticarial rash and monoclonal IgM gammopathy and is sometimes associated with periodic fever, arthralgias, and bone pain. Current treatment is unsatisfactory. OBSERVATIONS: Eleven patients with Schnitzler syndrome were treated with oral pefloxacin mesylate (800 mg/d). In 10 patients, we observed a dramatic and sustained improvement of urticarial and systemic manifestations. Corticosteroid therapy could be stopped or reduced in 6 patients. In 9 patients, pefloxacin was administered for more than 6 months (

Asunto(s)
Antiinfecciosos/uso terapéutico , Pefloxacina/uso terapéutico , Síndrome de Schnitzler/tratamiento farmacológico , Adulto , Anciano , Anciano de 80 o más Años , Antiinfecciosos/administración & dosificación , Estudios de Cohortes , Quimioterapia Combinada , Glucocorticoides/administración & dosificación , Humanos , Persona de Mediana Edad , Pefloxacina/administración & dosificación , Estudios Retrospectivos , Resultado del Tratamiento
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