Livedoid vasculopathy (LV) is a rare, chronic, and occlusive disease of the veins supplying the upper parts of the skin. The pathogenesis of the disease is not precisely understood, and its attacks are often unpredictable but tend to worsen during the summer. LV affects women more often. This increased risk for LV in women might be related to sex-specific physiological conditions, such as pregnancy, or a higher incidence of LV-associated conditions, such as connective tissue diseases, hypercoagulable states, and venous stasis in women. The typical clinical appearance of LV consists of three main findings: livedo racemose, atrophie blanche, and skin ulcers. The purpose of this comprehensive review was to analyze LV in all aspects and mainly focus on early diagnosis for successful clinical management with a holistic and multidisciplinary approach. A detailed history, dermatological examination, and laboratory testing are essential for a diagnosis of LV. When LV is clinically suspected, a skin biopsy should be taken to confirm the diagnosis. Another critical step is to investigate the underlying associated conditions, such as connective tissue diseases, hypercoagulable states, thrombophilia, and malignancy. Unfortunately, no associated conditions can be detected in approximately 20% of all cases (idiopathic LV) despite all efforts. The diagnosis of the disease is delayed in most patients. Thus, irreversible, permanent scars appear. Early and appropriate treatment reduces pain and prevents the development of scars and other complications. Antiplatelet drugs and anticoagulants can be preferred as the first-line treatments along with general supportive measures. Other therapeutic options might be considered in unresponsive cases. Preference for refractory cases is based on availability, clinical experience, and patient-related factors (comorbidities, age, sex, and compliance). These include anabolic steroids, intravenous immunoglobulin, hyperbaric oxygen therapy, psoralen-ultraviolet A, vasodilators, fibrinolytics, immunomodulators, and immunosuppressives.

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Behcet’s disease (BD) is a chronic systemic vasculitis with inflammation attacks that involve multiple organs. In addition to numerous mucocutaneous symptoms, notably recurrent oral and genital ulcers, ocular, articular, vascular, gastrointestinal, cardiac, and neurological system involvement can be observed. Mucocutaneous lesions are the primary symptom of the disease in most patients, and they usually occur before major organ involvement and other symptoms of the disease. Recognizing the disease’s mucocutaneous lesions is very important to diagnose at an early stage, control with appropriate treatment and close follow-up, and prevent major organ involvement. Genome-wide association studies (GWAS) in recent years have confirmed that HLA-B*51 is the most significant genetic predisposing factor. The majority of gene polymorphisms have been detected in molecules that respond to microorganisms and genes encoding cytokines and adhesion molecules. The infectious agent S. sanguinis -commonly found in the oral mucosa of patients with BD- or the differences in the salivary or intestinal microbiome composition can trigger innate immune-mediated inflammation sustained by acquired or adaptive immune responses. In antigen-presenting cells (APCs), epistatic interactions between HLA-B*51 and endoplasmic reticulum aminopeptidase 1 (ERAP1) variants lead to the disruption of T-cell homeostasis, especially the activation of Type1 T-helper and Th17 pathway and suppression of regulatory T-cells. Recent developments to clarify the disease’s etiopathogenesis provided us with a better understanding of the mechanism of action of the relatively old drugs while opening a way for many new treatment methods. Apremilast has become an important option in the treatment of mucocutaneous symptoms with its high efficacy and safety. The disease increases the mortality rate, especially in young male patients. New treatments, especially anti-TNF-α agents, have provided significant progress and decreased the mortality rates with their rapid effect and high efficacy in patients with severe organ involvement and resistance to traditional immunosuppressive and immunomodulatory therapies. The use of IL-1, IL-6, IL-17, IL-12/IL-23 antagonists in different organ involvement has gradually increased, and the quality of life has significantly improved in many patients.

Objective The aim of this multi‐center study was to assess predictive factors for work‐day loss as an indirect cost element in Behçet's syndrome (BS). Methods In this cross‐sectional, multi‐center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self‐reported work‐day loss during the previous year. Results Work‐day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration <5 years (74%) were higher in male patients with work‐day loss (P < .05). The majority of males (90.9%) had more than four clinic visits during the previous year. Moreover, the mean work‐day loss (30.8 ± 57.7 days) was higher in patients with vascular involvement (56.1 ± 85.9) than those without (26.4 ± 50.6 days) (P = .046). In addition, increased frequency of ocular involvement (25.9%) was also observed in patients with work‐day loss compared to others (8.6%) (P = .059). Conclusion Work‐day loss was associated with both vascular and ocular involvement. Close associations were observed among male gender, early period of the disease, frequent medical visits, being a smoker and treatment with IS medications in patients with work‐day loss.