Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 11 de 11
Filtrar
Mais filtros










Base de dados
Intervalo de ano de publicação
1.
Pediatr Dermatol ; 36(6): 984-985, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31448438

RESUMO

Ocular mucous membrane pemphigoid (MMP) is a chronic autoimmune blistering disease that usually affects elderly patients being extremely rare in pediatric population. Despite aggressive immunosuppressive therapy, ocular MMP may progress causing significant morbidity. Herein, we describe a toddler with ocular MMP successfully treated with rituximab.

2.
Front Med (Lausanne) ; 5: 268, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30320114

RESUMO

An 83-year-old patient developed erosions and a blister of the gingival mucous membrane, 6 months after discontinuation of the anti-programmed death-1 (anti PD-1) pembrolizumab therapy administered for 10 months for a metastatic melanoma. A diagnosis of mild mucous membrane pemphigoid (MMP) was made. Complete remission of MMP was rapidly obtained with minimal therapy (doxycycline). MMP remained in complete remission after a 3-month follow-up since discontinuation of the doxycycline therapy and no evidence of relapse of the melanoma was observed after a 14-month follow-up since discontinuation of the pembrolizumab therapy. The widespread use of anti PD-1 and anti-programmed death-ligand-1 (PD-L1) in several malignancies reveals new adverse events. MMP describes a group of chronic, inflammatory, mucous membrane-predominant, subepithelial auto-immune blistering diseases. It is clinically distinct from bullous pemphigoid another autoimmune blistering disease but shares some immunological similarities with it. Twenty-nine cases of bullous pemphigoid associated with anti PD-1/PD-L1 have been reported in the literature and one of MMP. Here, we described the case of a MMP developed after pembrolizumab and discussed the accountability of anti PD-1/PD-L1 in our case and the previous reported bullous pemphigoid and MMP cases using the Begaud system scoring.

3.
Front Immunol ; 9: 1030, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29881377

RESUMO

Mucous membrane pemphigoids (MMPs) and bullous pemphigoid (BP) are autoimmune bullous diseases that share physiopathological features: both can result from autoantibodies directed against BP180 or BP230 antigens. An association has been reported between BP and intake of gliptins, which are dipeptidyl peptidase-IV inhibitors used to treat type 2 diabetes mellitus. Clinical and immunological differences have been reported between gliptin-induced BPs and classical BPs: mucosal involvement, non-inflammatory lesions, and target BP180 epitopes other than the NC16A domain. Those findings accorded gliptins extrinsic accountability in triggering MMP onset. Therefore, we examined gliptin intrinsic accountability in a cohort of 313 MMP patients. To do so, we (1) identified MMP patients with gliptin-treated (challenge) diabetes; (2) selected those whose interval between starting gliptin and MMP onset was suggestive or compatible with gliptin-induced MMP; (3) compared the follow-ups of patients who did not stop (no dechallenge), stopped (dechallenge) or repeated gliptin intake (rechallenge); (4) compared the clinical and immunological characteristics of suggestive-or-compatible-challenge patients to 121 never-gliptin-treated MMP patients serving as controls; and (5) individually scored gliptin accountability as the trigger of each patient's MMP using the World Health Organization-Uppsala Monitoring Center, Naranjo- and Begaud-scoring systems. 17 out of 24 gliptin-treated diabetic MMP patients had suggestive (≤12 weeks) or compatible challenges. Complete remission at 1 year of follow-up was more frequent in the 11 dechallenged patients. One rechallenged patient's MMP relapsed. These 17 gliptin-treated diabetic MMP patients differed significantly from the MMP controls by more cutaneous, less buccal, and less severe involvements and no direct immunofluorescence IgA labeling of the basement membrane zone. Multiple autoantibody-target antigens/epitopes (BP180-NC16A, BP180 mid- and C-terminal parts, integrin α6ß4) could be detected, but not laminin 332. Last, among the 24 gliptin-treated diabetic MMP patients, five had high (I4-I3), 12 had low (I2-I1) and 7 had I0 Begaud intrinsic accountability scores. These results strongly suggest that gliptins are probably responsible for some MMPs. Consequently, gliptins should immediately be discontinued for patients with a positive accountability score. Moreover, pharmacovigilance centers should be notified of these events.

4.
Semin Arthritis Rheum ; 48(1): 83-89, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29191376

RESUMO

BACKGROUND: Bullous systemic lupus erythematosus (BSLE) is a rare blistering condition associated with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: We conducted a multi-center retrospective study and literature review in order to describe the clinical, immunological, and histological presentations and outcomes of BSLE. The skin biopsies were centrally reviewed, and sera obtained during a flare of BSLE were analyzed for identification of circulating anti-basement membrane zone antibodies. RESULTS: Ten patients (all women, median age at SLE diagnosis of 22 years) were included, as well as 118 cases from a systematic review of the literature. Lupus nephritis was associated in 50% of the cases. BSLE presented as tensed bullae on normal or erythematous skin, predominantly localized on the trunk, arms, head, and neck. Urticarial lesions were associated in 31% of the cases, and mucous membrane involvement was seen in 51%. Histological analyses displayed subepidermal detachment, dermal infiltration of polynuclear neutrophils, alignment of these cells at the basal membrane zone and leukocytoclasis. The direct immunofluorescence was polymorphic, showing linear and/or granular deposits of IgG, IgA, IgM, and/or C3. Anti-type VII collagen antibodies were detected in 69% of cases. Dapsone was efficacious in 90% of cases. CONCLUSION: BSLE is rather an autoimmune neutrophilic blistering disease associated with SLE than a cutaneous manifestation and may be associated with active extra-cutaneous manifestations of SLE. Dapsone is the first-choice option.


Assuntos
Vesícula/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Pele/patologia , Adulto , Anti-Infecciosos , Vesícula/tratamento farmacológico , Vesícula/patologia , Dapsona/uso terapêutico , Feminino , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/patologia , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
5.
J Am Acad Dermatol ; 74(6): 1166-72, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-26947449

RESUMO

BACKGROUND: Serologic diagnosis of epidermolysis bullosa acquisita (EBA) relies on the detection of circulating autoantibodies to type VII collagen (C7). OBJECTIVE: We sought to compare the diagnostic performances of a commercialized enzyme-linked immunosorbent assay (ELISA) using C7 noncollagenous (NC) domains (C7-NC1/NC2 ELISA) and indirect immunofluorescence (IIF) biochip test on NC1-C7-expressing transfected cells (IIFT), with a full-length-C7 ELISA developed in our laboratory. METHODS: C7-NC1/NC2 ELISA, IIFT, and full-length-C7 ELISA were run on 77 nonselected consecutive EBA sera. RESULTS: C7-NC1/NC2 ELISA, IIFT, and full-length-C7 ELISA were positive, respectively, for: 30%, 27%, and 65% of the 77 sera; 43%, 32%, and 80% of 44 sera labeling the salt-split-skin (SSS) floor (F) by IIF (SSS/F(+)); 9%, 22%, and 47% of 32 SSS/F(-) sera; 28%, 28%, and 58% of classic EBA; 41%, 41%, and 82% of inflammatory EBA; and 18%, 0%, and 55% of mucous-membrane-predominant EBA. Significant differences for all sera were found between: the 2 ELISAs for the 77 sera, SSS/F(+) and SSS/F(-) sera, and IIFT versus full-length-C7 ELISA. LIMITATIONS: The retrospective design was a limitation. CONCLUSION: C7-NC1/NC2 ELISA and IIFT sensitivities for serologic diagnoses of EBA were low. Full-length-C7 ELISA was significantly more sensitive and could serve as a reference test.


Assuntos
Autoanticorpos/sangue , Colágeno Tipo VII/imunologia , Epidermólise Bolhosa Adquirida/sangue , Epidermólise Bolhosa Adquirida/diagnóstico , Testes Sorológicos/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Área Sob a Curva , Criança , Pré-Escolar , Ensaio de Imunoadsorção Enzimática/métodos , Feminino , Técnica Indireta de Fluorescência para Anticorpo , Humanos , Masculino , Pessoa de Meia-Idade , Análise Serial de Proteínas , Curva ROC , Estudos Retrospectivos , Adulto Jovem
6.
Br J Haematol ; 168(5): 671-8, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25363150

RESUMO

This retrospective analysis was conducted in 64 patients diagnosed with type I cryoglobulinaemia (CG) followed at two French centres. Median follow-up was 6·75 years. CG was IgG in 60% and IgM in 40% of all cases and was asymptomatic in 16 patients (25%). Cold-triggered ischaemic skin manifestations were observed in 33 patients (51%). Neurological manifestations were observed in 15 patients and renal manifestations in 13. Most of the patients with necrotic purpura (14/16, P = 0·009) and renal manifestations (11/13, P = 0·057) had IgG CG. IgG CG was associated with monoclonal gammopathy of undetermined significance (MGUS), myeloma, chronic lymphocytic leukaemia and lymphoplasmocytic lymphoma in 18, 13, 5 and 2 patients, respectively. IgM CG was associated with MGUS and Waldenström macroglobulinaemia in 8 and 18 cases, respectively. One third of patients did not receive any specific treatment. Various treatments, including rituximab, were administered to 25/31 patients with IgG CG and 6/25 patients with IgM CG due to CG-related symptoms. Rituximab was ineffective in all cases associated with a predominantly plasmacytic proliferation. To conclude, type I CG has specific clinico-biological characteristics compared to type II CG. Furthermore, there are differences in terms of related manifestations between type I IgG and type I IgM CG.


Assuntos
Anticorpos Monoclonais Murinos/administração & dosagem , Antineoplásicos/administração & dosagem , Crioglobulinemia , Imunoglobulina G/sangue , Paraproteinemias , Macroglobulinemia de Waldenstrom , Idoso , Anticorpos Monoclonais Murinos/efeitos adversos , Antineoplásicos/efeitos adversos , Crioglobulinemia/sangue , Crioglobulinemia/tratamento farmacológico , Crioglobulinemia/patologia , Feminino , Seguimentos , Humanos , Isquemia/sangue , Isquemia/tratamento farmacológico , Isquemia/patologia , Rim/patologia , Nefropatias/sangue , Nefropatias/tratamento farmacológico , Nefropatias/patologia , Masculino , Paraproteinemias/sangue , Paraproteinemias/tratamento farmacológico , Paraproteinemias/patologia , Estudos Retrospectivos , Rituximab , Pele/irrigação sanguínea , Pele/patologia , Dermatopatias/sangue , Dermatopatias/tratamento farmacológico , Dermatopatias/patologia , Macroglobulinemia de Waldenstrom/sangue , Macroglobulinemia de Waldenstrom/tratamento farmacológico , Macroglobulinemia de Waldenstrom/patologia
7.
Transfusion ; 54(8): 1988-95, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-24697848

RESUMO

BACKGROUND: We describe a patient with a high-titer warm immunoglobulin (Ig)A autoantibody resulting in death due to hemagglutination rather than to hemolysis. CASE REPORT: A 47-year-old male patient presented with an intriguing pronounced vascular erythema of the skin. A livedo reticularis associated with cold agglutinin of high thermal amplitude was suspected. The patient's condition unexpectedly and abruptly deteriorated resulting in death 3 days after admission. STUDY DESIGN AND METHODS: Conventional serologic procedures and immunochemical methods were used. RESULTS: Serologic and immunochemical examinations revealed a warm IgA autoantibody of high titer with anti-Band 3 specificity. Although the patient presented with severe anemia, only mild signs of hemolysis were observed, with no evidence of complement activation. The autopsy revealed an enormous accumulation of agglutinated red blood cells in liver and spleen and a B-cell lymphoma and cerebral edema. Thus, the patient's death was largely caused by hypoxia related to hemagglutination rather than to hemolysis and/or anemia per se. CONCLUSION: Strongly hemagglutinating antibodies may not only cause immune hemolysis but also hypoxia due to intravascular hemagglutination.


Assuntos
Anemia/imunologia , Proteína 1 de Troca de Ânion do Eritrócito/imunologia , Autoanticorpos/imunologia , Autoantígenos/imunologia , Doenças Autoimunes/imunologia , Hemaglutinação , Hemaglutininas/imunologia , Imunoglobulina A/imunologia , Livedo Reticular/etiologia , Motivos de Aminoácidos , Anemia/sangue , Anemia/complicações , Anemia/diagnóstico , Anemia Hemolítica Autoimune/diagnóstico , Proteína 1 de Troca de Ânion do Eritrócito/química , Especificidade de Anticorpos , Autoanticorpos/sangue , Doenças Autoimunes/sangue , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Edema Encefálico/etiologia , Diagnóstico Diferencial , Evolução Fatal , Hemaglutininas/sangue , Humanos , Hipóxia/etiologia , Imunoglobulina A/sangue , Achados Incidentais , Fígado/patologia , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/imunologia , Masculino , Pessoa de Meia-Idade , Baço/patologia
9.
J Invest Dermatol ; 131(12): 2386-93, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21833018

RESUMO

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune bullous disease (AIBD). However, higher EBA incidence and predisposing genetic factor(s) involving an HLA haplotype have been suspected in some populations. This retrospective study assessed the overrepresentation of black patients with EBA, its link with HLA-DRB1*15:03, and their clinical and immunological characteristics. Between 2005 and 2009, 7/13 (54%) EBA and 6/183 (3%) other-AIBD patients seen consecutively in our department were black (P=10(-6)); moreover 7/13 (54%) black patients and 6/183 (3%) white patients had EBA (P=10(-6)). In addition, between 1983 and 2005, 12 black patients had EBA. Finally, among the 19 black EBA patients, most of them had very atypical clinical presentations, 9 were natives of sub-Saharan Africa, 1 from Reunion Island, 7 from the West Indies, and 2 were of mixed ancestry. HLA-DRB1*15:03 allelic frequencies were 50% for African patients, significantly higher than the control population (P<10(-3)), and 21% for the West Indians (nonsignificant). High EBA frequencies have already been reported in American blacks significantly associated with the HLA-DR2. In conclusion, black-skinned patients developing EBA seem to have a genetic predisposition, and EBA should be suspected systematically for every AIBD seen in this population.


Assuntos
Grupo com Ancestrais do Continente Africano/genética , Epidermólise Bolhosa Adquirida/genética , Frequência do Gene , Cadeias HLA-DRB1/genética , Adolescente , Adulto , Grupo com Ancestrais do Continente Africano/estatística & dados numéricos , Criança , Pré-Escolar , Epidermólise Bolhosa Adquirida/epidemiologia , Grupo com Ancestrais do Continente Europeu/genética , Grupo com Ancestrais do Continente Europeu/estatística & dados numéricos , Feminino , Predisposição Genética para Doença , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
10.
Arch Dermatol ; 147(7): 843-9, 2011 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-21422323

RESUMO

BACKGROUND: Mucous membrane pemphigoid (MMP) still represents a potentially life- and sight-threatening disease. In a subset of patients with severe MMP, conventional immunosuppressants are ineffective or contraindicated. OBSERVATIONS: Twenty-five patients with severe refractory MMP, including 5 with mucous membrane-dominant epidermolysis bullosa acquisita, received 1 or 2 cycles of rituximab (375 mg/m(2) weekly for 4 weeks). Twenty-one of the patients were receiving concomitant therapy with dapsone and/or sulfasalazine therapy, which was maintained during rituximab cycles. Complete responses in all affected sites (ocular and/or extraocular) were obtained in 17 patients (68%) by a median time of 12 weeks after the first cycle, and 5 additional patients responded completely after a second cycle, yielding an 88% complete response rate. In all but 1 of the 10 patients with ocular lesions, their eyes became noninflammatory within a mean of 10 weeks. Among the 3 patients (12%) who developed severe infectious complications, 2 (8%) died; they had been receiving concomitant conventional immunosuppressants and high-dose corticosteroids and were hypogammaglobulinemic. Treatment with immunosuppressants was discontinued for all other patients, and no other infection was observed. Ten patients experienced relapse after a mean of 4 (range, 1-16) months after achieving complete responses. CONCLUSIONS: Rituximab appears to have rapid and dramatic efficacy in patients with severe, refractory MMP. The occurrence of severe infections in patients receiving concomitant conventional immunosuppressants supports using rituximab without other immunosuppressants. Controlled prospective studies are warranted to define an optimal treatment protocol.


Assuntos
Anticorpos Monoclonais Murinos/uso terapêutico , Fatores Imunológicos/uso terapêutico , Penfigoide Mucomembranoso Benigno/tratamento farmacológico , Adolescente , Adulto , Agamaglobulinemia/induzido quimicamente , Agamaglobulinemia/imunologia , Idoso , Idoso de 80 Anos ou mais , Anticorpos Monoclonais Murinos/imunologia , Autoanticorpos/imunologia , Estudos de Coortes , Doenças Transmissíveis/induzido quimicamente , Doenças Transmissíveis/imunologia , Dapsona/imunologia , Dapsona/uso terapêutico , Fármacos Dermatológicos/imunologia , Fármacos Dermatológicos/uso terapêutico , Feminino , Humanos , Fatores Imunológicos/imunologia , Imunossupressores/efeitos adversos , Imunossupressores/imunologia , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Penfigoide Mucomembranoso Benigno/imunologia , Rituximab , Índice de Gravidade de Doença , Sulfassalazina/imunologia , Sulfassalazina/uso terapêutico , Resultado do Tratamento , Adulto Jovem
11.
Arch Dermatol ; 140(1): 91-6, 2004 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-14732665

RESUMO

BACKGROUND: Pemphigus vulgaris (PV) is a severe antibody-mediated autoimmune blistering disease. Because some patients with PV do not enter into remission, despite the use of high-dose corticosteroid therapy and immunosuppressive adjuvant treatments, new effective and safer agents are warranted to treat refractory PV. Rituximab, a monoclonal anti-CD20 antibody, induces depletion of B cells in vivo and has shown efficacy in patients with refractory antibody-mediated autoimmune disorders. We describe herein 3 patients treated with rituximab for severe PV. OBSERVATIONS: Three patients with refractory PV were treated with rituximab, resulting in a clinical response in all patients, which was complete in 2 patients. A decline in titers of circulating antiepidermis autoantibodies paralleled disease activity, while circulating B cells remained undetectable for 6 to 10 months. Two patients experienced bacterial infection in the weeks following the rituximab course. A clinical relapse occurred in 2 patients, at 6 and 10 months. A second course of rituximab controlled the disease in one of them. CONCLUSION: These patients' response suggests that rituximab may be a valuable treatment for refractory PV and warrants further studies to evaluate the risk-benefit ratio in patients with PV showing resistance to classic therapy.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Pênfigo/tratamento farmacológico , Adulto , Anticorpos Monoclonais Murinos , Autoanticorpos/sangue , Epiderme/imunologia , Feminino , Humanos , Masculino , Pênfigo/imunologia , Pênfigo/patologia , Rituximab
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA