Tumor necrosis factor‐like weak inducer of apoptosis and its receptor fibroblast growth factor‐inducible 14 are expressed in urticarial vasculitis

Tumor necrosis factor (TNF)‐like weak inducer of apoptosis (TWEAK), a member of the TNF family, has been implicated as a pro‐inflammatory cytokine in many types of autoimmune and infectious diseases. However, information about TWEAK in dermatological diseases is limited. To date, no studies have investigated the roles of TWEAK in patients with urticarial vasculitis (UV). This study aimed to assess serum TWEAK levels, together with TWEAK and fibroblast growth factor‐inducible 14 (Fn14) expressions of skin lesions in patients with UV. Serum TWEAK levels in patients with UV, together with patients with cutaneous leukocytoclastic angiitis (CLA) and healthy controls were detected by enzyme‐linked immunosorbent assay; TWEAK and Fn14 expressions of skin lesions were analyzed by immunohistochemistry. Results showed that TWEAK and Fn14 were abundantly expressed in the dermal vessel wall of lesional skin in patients with UV but not healthy controls. Serum TWEAK levels in the acute stage in patients with UV were significantly higher than those in the convalescent stage and healthy controls. Serum TWEAK levels were elevated significantly in patients with CLA compared with those in healthy controls. Our previous study indicated that TWEAK may be an important mediator for the development of vascular inflammation in skin. In addition, we also found that TWEAK blockade substantially reduced vascular damage and perivascular leukocyte infiltrates in lipopolysaccharide‐induced cutaneous vasculitis. Our study shows that TWEAK may be associated with the pathogenesis of UV; it is therefore suggested that TWEAK may be a potential therapeutic target for UV and other types of cutaneous vasculitis.     

Blepharoptosis in association with ipsilateral adduction and elevation palsy A form of fascicular oculomotor palsy

A 16-year-old girl hospitalized with a sudden onset of blepharoptosis and diplopia revealed a combined paresis of the elevator palpebrae, the superior rectus, and the medial rectus muscles of the left eye as quantitatively demonstrated by the Hess chart and levator action test. A small midbrain lesion confined to the left cerebral peduncle and tegmentum, presumably by an ischemic or demyelinative process, was identified on magnetic resonance imaging. Despite uncertainty in its pathology, the incomplete oculomotor palsy of this case is certainly a consequence of oculomotor fascicular involvement in the intra-axial nerve root which initially fans out and then converges into the peripheral compact bundle. We speculate on the revised version of the oculomotor fascicular arrangements by which the neurophthalmologic features of the current case are better explained.     

Anti-endothelial cell antibodies (AECA) in patients with systemic vasculitis: our research using proteomics

Importance of the field: Anti-endothelial cell antibodies (AECA) may cause damage to endothelial cell (EC) functions and therefore may be of a pathophysiological role in systemic vasculitis. The pathophysiological role of AECA, however, is still uncertain.

Areas covered in this review: To clarify the detailed roles of AECA, various methods for identification of target proteins of AECA have been developed, such as expression libraries and proteomic approaches combining two-dimensional electrophoresis and immunoblotting.

What the reader will gain: Advances, including our research, have been made in defining the target antigens of AECA, which we summarize in this review. Furthermore, we discuss the possible significance of AECA in the pathophysiology of vascular damage and the value of AECA in systemic vasculitis.

Take home message: To identify target antigens of AECA and to establish a standardized method for measuring AECA would be helpful in the search for a possible pathophysiological role of AECA in systemic vasculitis.     

Urticarial vasculitis after meningococcal serogroup B vaccine in a 6‐year‐old girl

The first vaccine that shows significant potential in providing broad coverage against serogroup B meningococcal disease has recently been approved. Because of its newness, potential adverse events need to be reported. Here we report a case of urticarial vasculitis, a rare disease in children, in probable relationship with the novel vaccine.     

Chilblain-like lesions in children following suspected COVID-19 infection

During the COVID-19 pandemic, chilblain-like lesions have been reported in mildly symptomatic children and adolescents. We present four children investigated for suspected COVID-19 infection who presented with acral skin findings and mild systemic symptoms. Histology from one case showed signs of vasculitis with evident fibrin thrombus.     

Interstitial granulomatous dermatitis and granulomatous arteritis in the setting of PD-1 inhibitor therapy for metastatic melanoma

Checkpoint inhibition has become an important target in the management of malignant melanoma. As anti-CTLA4 inhibitors and anti-PD1 antibodies are increasingly utilized, reports of immune-related adverse events (IRAEs) are becoming more frequent. Common noted cutaneous IRAEs are morbilliform, lichenoid, bullous, granulomatous, psoriasiform, and eczematous eruptions. We report a case of interstitial granulomatous dermatitis and granulomatous arteritis in the setting of nivolumab (anti-PD1) monotherapy for metastatic melanoma. There are many different causes for granulomatous vasculitis, such as herpes virus infection, lymphoproliferative disorders, systemic vasculitis, and inflammatory bowel disease. This report adds to the growing literature on granulomatous IRAEs due to checkpoint inhibition.     

Predicting Mortality in Microscopic Polyangiitis with Renal Involvement: A Survival Analysis Based on 64 Patients

Background: To determine predictors of survival in patients with microscopic polyangiitis (MPA). Methods: A cohort of 64 patients who met the Chapel Hill criteria for MPA with renal involvement participated in the study. All subjects received cytotoxic drugs. All of the diagnoses were biopsy proven. Results: We retrospectively studied 64 patients (median age, 59 years; male/female ratio, 1.6:1). The mean follow-up was 38 months; 34 (53.13%) patients died or acquired end-stage renal disease. According to univariate analysis, a preliminary prognostic value was attributed to serum creatinine (Scr) > 459 μmol/L (p < 0.001); erythrocyte sedimentation rate (ESR) > 99 mm/h (p < 0.001); serum albumin < 30 g/L (p < 0.001); and hemoglobin < 84 g/L (p < 0.001). Logistic regression analysis showed that Scr level (β = 1.02, p = 0.0002) and ESR (β = 1.02, p = 0.0002) at baseline were associated with poor prognosis, and Cox regression analysis further confirmed this result [Scr: β = 1.004, 95% confidence interval (CI): 1.002–1.006, p < 0.001; ESR: β = 1.018, 95% CI: 1.000–1.037, p = 0.046]. The receiver operating characteristic curve showed that Scr and ESR were predictors of MPA patient prognosis, their areas under the curves were 0.95 and 0.80, their sensitivities were 94.1% and 92.3%, and their specificities were 94% and 70%, respectively. Conclusion: Despite the small number of patients in this study, the prevalence of renal vasculitis was high in patients with MPA. The level of Scr and ESR may be a useful clinical biomarker for monitoring prognosis.