RESUMO
OBJECTIVES: To describe the clinical and pathological features of biopsy-proven cutaneous vasculitis (CV) associated with SLE, focusing on diagnosis classification and impact on overall SLE activity. METHODS: Retrospective multicentric cohort study including SLE patients with biopsy-proven CV identified by (i) data from pathology departments of three university hospitals and (ii) a national call for cases. SLE was defined according to 1997 revised ACR and/or 2019 ACR/EULAR criteria. CV diagnosis was confirmed histologically and classified by using the dermatological addendum of the Chapel Hill classification. SLE activity and flare severity at the time of CV diagnosis were assessed independently of vasculitis items with the SELENA-SLEDAI and SELENA-SLEDAI Flare Index. RESULTS: Overall, 39 patients were included; 35 (90%) were female. Cutaneous manifestations included mostly palpable purpura (n = 21; 54%) and urticarial lesions (n = 18; 46%); lower limbs were the most common location (n = 33; 85%). Eleven (28%) patients exhibited extracutaneous vasculitis. A higher prevalence of Sjögren's syndrome (51%) was found compared with SLE patients without CV from the French referral centre group (12%, P < 0.0001) and the Swiss SLE Cohort (11%, P < 0.0001). CV was mostly classified as urticarial vasculitis (n = 14, 36%) and cryoglobulinaemia (n = 13, 33%). Only 2 (5%) patients had no other cause than SLE to explain the CV. Sixty-one percent of patients had inactive SLE. CONCLUSION: SLE-related vasculitis seems very rare and other causes of vasculitis should be ruled out before considering this diagnosis. Moreover, in more than half of patients, CV was not associated with another sign of active SLE.
Assuntos
Lúpus Eritematoso Sistêmico , Dermatopatias Vasculares , Urticária , Vasculite , Humanos , Feminino , Masculino , Estudos Retrospectivos , Estudos de Coortes , Lúpus Eritematoso Sistêmico/diagnóstico , Dermatopatias Vasculares/etiologia , Vasculite/complicações , Urticária/complicaçõesAssuntos
Glucocorticoides/uso terapêutico , Leucemia Mieloide Aguda/complicações , Síndromes Mielodisplásicas/complicações , Síndrome de Sweet/epidemiologia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Leucemia Mieloide Aguda/sangue , Leucemia Mieloide Aguda/imunologia , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/sangue , Síndromes Mielodisplásicas/imunologia , Recidiva , Estudos Retrospectivos , Fatores de Risco , Pele/imunologia , Pele/patologia , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/tratamento farmacológico , Síndrome de Sweet/imunologia , Exacerbação dos Sintomas , Fatores de TempoAssuntos
Leucemia Mieloide Aguda/complicações , Síndromes Mielodisplásicas/complicações , Transtornos Mieloproliferativos/complicações , Neutrófilos/imunologia , Síndrome de Sweet/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Medula Óssea/imunologia , Medula Óssea/patologia , Hematopoiese Clonal/imunologia , Estudos de Coortes , Análise Mutacional de DNA , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Leucemia Mieloide Aguda/sangue , Leucemia Mieloide Aguda/genética , Leucemia Mieloide Aguda/imunologia , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/sangue , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/imunologia , Transtornos Mieloproliferativos/sangue , Transtornos Mieloproliferativos/genética , Transtornos Mieloproliferativos/imunologia , Pele/citologia , Pele/imunologia , Pele/patologia , Síndrome de Sweet/sangue , Síndrome de Sweet/genética , Síndrome de Sweet/patologia , Adulto JovemRESUMO
Varicella zoster virus (VZV) primo-infection can be severe in the elderly and in immunocompromised patients. Atypical presentations are not uncommon and may mislead the diagnosis and delay adequate treatment. Valacyclovir prophylaxis should be systematically proposed in immunocompromised patients.
RESUMO
BACKGROUND: Anti-tumor-necrosis-factor-alpha agents are limited by their side effects. Eczema is one of the most frequent adverse reactions affecting quality of life. OBJECTIVE: To assess potential predictive risk factors for eczema in patients receiving infliximab. METHODS: We conducted a prospective cohort study including patients treated with infliximab for a variety of disorders with the exception of cutaneous psoriasis. Clinical features were compared among patients with and without eczema under therapy. RESULTS: 92 consecutive patients were included; 15 developed eczema after the initiation of infliximab. In univariate analyses, a personal history of atopic symptoms was the only predictive factor for the occurrence of eczema (odds ratio = 3.6). Sex, age, principal diagnosis, dose and duration of infliximab and concomitant use of other immunosuppressors had no influence on the occurrence of eczema. CONCLUSIONS: A personal history of atopic symptoms is predictive of eczema under infliximab. Specific information should be provided to atopic patients starting such a treatment.
Assuntos
Anti-Inflamatórios/efeitos adversos , Anticorpos Monoclonais/efeitos adversos , Toxidermias/patologia , Eczema/induzido quimicamente , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Inflamatórios/administração & dosagem , Anticorpos Monoclonais/administração & dosagem , Artrite Reumatoide/tratamento farmacológico , Biópsia , Doença de Crohn/tratamento farmacológico , Diagnóstico Diferencial , Eczema/patologia , Feminino , Seguimentos , Humanos , Infliximab , Infusões Intravenosas , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Psoríase , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto JovemRESUMO
BACKGROUND: Extraintestinal manifestations of Crohn's disease may involve the skin, the eyes, the genital mucosa, and the joints. Dermatoses associated with Crohn's disease include neutrophilic dermatoses, erythema nodosum, granulomatous dermatitis, blistering dermatoses, and non-specific skin manifestations. Cutaneous Crohn's disease is characterized by skin non-caseating epithelioid granulomatas with giant cells, remote from the gastrointestinal tract. We report herein two new cases. OBSERVATIONS: On both patients, differential diagnosis of neutrophilic dermatoses and infectious disease were evoked, and antimicrobial agents were introduced in one of them. Given the atypical presentation, the final diagnosis of cutaneous Crohn's disease could only be made with histological examination. In patient 1, the plaques decreased in size and infiltration by more than 75% after 3 weeks of treatment with bethametasone dipropionate 0.05% cream. In patient 2, the plaques decreased by more than 50% after 6 weeks of treatment with prednisolone (45 mg/day) and azathioprine (100 mg/day). DISCUSSION: Cutaneous Crohn's disease may present as dusky, erythematous, infiltrated, and ulcerated plaques and nodules. Female-to-male sex ratio is about 2, and the mean age at onset is 35. Recurrently, the hypothesis of a skin mycobacterial or fungal infection greatly delays proper treatment. Rarity of cutaneous Crohn's disease hampers therapeutic assessment in controlled trials. Thus, available literature is limited to case reports and sparse small series, with contradictory results. These reports are subject to publication bias, and no definite evidence-based recommendations can be made on the most adequate therapeutic strategy.