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Behcet's disease: review with emphasis on dermatological aspects.

ABSTRACT: Behçet's disease is a systemic vasculitis characterized by attacks of acute inflammation, which can affect almost every vascularized area of the body. There is a close correlation between the geographical distribution of HLA-B51 and its prevalence. In the etiopathogenesis there are indications of genetic susceptibility associated with environmental influence. Among the involved genes are those that encompass innate and adaptive immunities. Polymorphisms and epistatic interactions in several genes are described, as well as the presence of imbalance lineage between HLA-B51 and A (MICA). Herpes simplex and Streptococcus sanguinis may be important extrinsic factors. An increase of Th1 response and of IL-21 is observed. The production of IL-21 is positively related to Th17 cells and negatively to T-regs. The mucocutaneous manifestations are Behcet´s disease markers, and their earlier onset indicates a worse prognosis. Recurrent oral ulcers have varied sizes and arrangements, genital ulcers are recurrent, leaving scars, skin lesions are multivaried, and pathergy, although not so frequent, is important for the diagnosis. There are numerous attempts to validate indexes that can evaluate the disease activity and among them the Mucocutaneous Activity Index. This is a specific score that can help with therapeutic decisions and to reduce morbidity, but still lacks validation. The clinical manifestations of other organs are described as well as treatment options.

SUBMITTER: Scherrer MAR

PROVIDER: S-EPMC5595589 | biostudies-literature | 2017 Jul-Aug

REPOSITORIES: biostudies-literature

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Behçet's disease: review with emphasis on dermatological aspects.

Scherrer Maria Antonieta Rios MAR Rocha Vanessa Barreto VB Garcia Lucas Campos LC

Anais brasileiros de dermatologia 20170701 4

Behçet's disease is a systemic vasculitis characterized by attacks of acute inflammation, which can affect almost every vascularized area of the body. There is a close correlation between the geographical distribution of HLA-B51 and its prevalence. In the etiopathogenesis there are indications of genetic susceptibility associated with environmental influence. Among the involved genes are those that encompass innate and adaptive immunities. Polymorphisms and epistatic interactions in several gene ...[more]

PMID: 28954091

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Project description:This study aimed to investigate the relationship between rosacea and headaches, focusing on different subtypes, as well as the associated clinical features and triggering factors. In this prospective study, 300 patients diagnosed with rosacea and 320 control subjects without rosacea or any connected mast cell activation illness were included. Patients with rosacea were assessed by a dermatologist according to the 2019 updated rosacea classification (ROSCO panel). Accordingly, patients were classified based on their predominant rosacea subtype as follows: erythematotelangiectatic (ETR), papulopustular (PPR), or phymatous (RhR). Patients experiencing headaches were assessed using the International Headache Classification. Headaches were categorized as migraine, tension-type headaches (TTHs), secondary types (STHs), and cluster-type headaches (CTHs). The ratio of headache was 30.3% in the rosacea group, which did not show a significant difference compared to the control group (30.3% vs. 25.0%, p = 0.138). In 81.3% of rosacea patients with headaches, headache onset occurred after the diagnosis of rosacea. The rate of patients with headaches was higher in the ETR group compared to the PPR and RhR groups (35.2% vs. 16.2% vs. 23.1%, p = 0.007, respectively). In terms of headache subtypes, the rates of patients with migraine and STHs were higher in the ETR group compared to the PPR and RhR groups, while the rate of patients with TTHs was higher in the RhR group. A positive correlation was found between rosacea severity and migraine severity (r = 0.284, p < 0.05). Among the triggering factors for rosacea, only sunlight was found to be associated with headaches. Lower age, female gender, and moderate to severe rosacea severity were identified as independent factors increasing the likelihood of headaches. A significant portion of rosacea patients experience headaches. Particularly, different subtypes of rosacea may be associated with various types of headaches. This study, highlighting the connection between migraine and ETR, is a pioneering work that demonstrates common pathogenic mechanisms and potential triggers.

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Behcet's disease: review with emphasis on dermatological aspects.

Publications

Behçet's disease: review with emphasis on dermatological aspects.

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