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Folliculosebaceous cystic hamartoma (FSCH) is a rare cutaneous hamartoma composed of dilated folliculosebaceous units associated with mesenchymal elements. Two cases of FSCH with typical histopathological features are reported. Patient 1 was a 60-year-old man presented with a normal skin-coloured asymptomatic nodule on his scalp. Patient 2 was a 70-year-old man with an asymptomatic nodule on his right auricle that had persisted for the previous 15 years. In all, 34 cases of FSCH have been reported in the English literature. Clinically, the lesions are asymptomatic, usually rubbery to firm in consistency, and usually occur on or above the neck (> 90%). Most lesions do not exceed 25 mm in diameter (> 90%). Histopathologically, FSCH shares several similar features to sebaceous trichofolliculoma, but it is usually possible to differentiate these two tumours.

This study aimed to determine whether ultrasonography (US) can detect increased vascular signal in the synovial tissue prior to overt synovitis in rheumatoid arthritis (RA). Env-pX rats that spontaneously develop RA-like synovitis were used. Ankle joints of 15 pre-morbid env-pX rats were observed with power Doppler and superb microvascular imaging (SMI) using an ultrahighfrequency (8-24 MHz) probe. Signal values were counted as the number of pixels. The total number of vessels and vessel area in the synovial tissue were histologically evaluated. Dilated vessels were determined from the mean value of synovial vessels in three wild-type rats. In all env-pX rats, apparent synovial proliferation was not observed. However, vasodilation was evident. Only SMI values were significantly correlated with the number of dilated vessels (r=0.585, p=0.022) but not with the total number of vessels. US with SMI using ultrahighfrequency probe can detect increased vascular signal in the synovial tissue of arthritis-prone rats.

Elevated plasma PSEP levels were correlated with disease activity of SLE, suggesting inappropriate monocyte or neutrophil activation in the pathophysiology of SLE exacerbation.

ObjectiveWe aimed to evaluate whether gadolinium-enhanced magnetic resonance imaging (MRI) in shoulders can contribute to more accurate diagnosis and prediction of recurrence in patients with polymyalgia rheumatica (PMR). Methods Gadolinium-enhanced MRI and ultrasonography (US) in shoulders were performed in the patients who had bilateral shoulders pain and fulfilled the Bird's Classification Criteria between June 2012 and June 2018. PMR was clinically diagnosed by at least two rheumatologists. MRI and US findings assessed by independent radiologists were comparedbetween the PMR or non-PMR patients. PMR patients were treated with 20 mg/day of prednisolone and were followed-up until June 2019 to determine any recurrences of the disease. Results PMR was diagnosed in 58 of 137 patients received gadolinium-enhanced MRI and US examinations. Enhancement of joint capsule, enhancement of rotator cuff tendon and focal bone oedema in humerus headswere frequently found in the PMR patients. If the three findings were used in combination to diagnose PMR, MRI had 76% sensitivity and 85% specificity, higher compared to US findings, which had 50% sensitivity and 72% specificity. During follow-up, PMR recurred in 24 patients. Patients with recurrent PMR were younger in age, had less enhancement of rotator cuff tendon and more synovial hypertrophy findings on their MRI. Conclusion Gadolinium-enhanced MRI could display capsulitis, rotator cuff tendonitis and focal bone oedema in humerus headsthat was sensitive and specific to patients with PMR, improving diagnostic accuracy in PMR. Rotator cuff tendonitis and synovial hypertrophy on MRI could help predict recurrence in PMR.

We report a case of epidermolysis bullosa acquisita (EBA) associated with psoriasis vulgaris. A 71-year-old woman with psoriasis vulgaris developed subepidermal blisters on the extremities. Direct immunofluorescence demonstrated linear deposit of IgG at the basement membrane zone, which bound to the dermal side of normal human skin split with 1 mol/L NaCl. Immunoblot analysis using recombinant full-length type VII collagen detected a 290-kDa band, confirming the diagnosis of EBA. A literature search for previous reports found a few cases of EBA associated with psoriasis, and all cases, including our own, presented with widespread inflammatory vesicles and bullae, and responded to conventional therapy with corticosteroids and immunosuppressive agents. This study suggests that western blotting using recombinant full-length type VII collagen could be useful for diagnosis of EBA, and that EBA associated with psoriasis may have a tendency to be the inflammatory type.

NKT cells are defined as T cells that recognize hydrophobic antigens presented by class I MHC-like molecules, including CD1d. Among CD1d-restricted NKT cells, type I and type II subsets have been noted. CD1d-restricted type I NKT cells are regarded as proinflammatory cells in general. On the contrary, accumulated evidence has demonstrated an anti-inflammatory property of CD1d-restricted type II NKT cells. In our earlier study using a rat model with vasculitis, we demonstrated the pro-inflammatory function of CD1d-restricted type II NKT cells and identified that one such cell recognized P 518-532 of rat sterol carrier protein 2 (rSCP2 518-532 ), which appeared on vascular endothelial cells presented by CD1d. Based on this evidence, we attempted to detect human CD1d-restricted type II NKT cells in peripheral blood using hSCP2 518-532 , the human counterpart of rSCP2 518-532, together with a CD1d tetramer in flow cytometry. First, we determined the binding of hSCP2 518-532 to CD1d. Next, we detected CD3-positive hSCP2 518-532 -loaded CD1d (hSCP2 518-532 /CD1d) tetramer-binding cells in peripheral blood of healthy donors. The abundance of TGF-β-producing cells rather than TNF-α-producing cells in CD3-positive hSCP2 518-532 /CD1d tetramer-binding cells suggests the anti-inflammatory property of SCP2-loaded CD1d (SCP2/CD1d) tetramer-binding type II NKT cells in healthy individuals. Furthermore, we compared cytokine profile between healthy individuals and patients with vasculitis in a pilot study. Interestingly, the percentage of TGF-β-producing cells in SCP2/CD1d tetramerbinding type II NKT cells in vasculitic patients was significantly lower than that in healthy controls despite the greater number of these cells. Although further studies to clarify the mechanism and significance of this phenomenon are needed, SCP2/CD1d tetramer-binding type II NKT cells in peripheral blood should be examined in more detail to understand the pathophysiology of vasculitides in humans.

We investigated the serum haptoglobin levels in patients with pulmonary arterial hypertension (PAH) based on the hypothesis that haptoglobin levels would reflect subclinical hemolysis due to microangiopathy in pulmonary arterioles.This cross-sectional study included 3 groups of patients attending Hokkaido University Hospital: PAH, chronic thromboembolic pulmonary hypertension (CTEPH), and connective tissue diseases (CTD) without PAH (CTD-non-PAH) group. Serum haptoglobin levels were measured by standardized turbidimetric immunoassay in all patients. Demographic data, laboratory results, right heart catheter, and echocardiographic findings were extracted from the medical records. Decreased haptoglobin levels were defined as below 19 mg/dL based on the 95th percentile of healthy controls.Thirty-five patients in PAH group including 11 with idiopathic PAH (IPAH) and 24 with CTD-associated PAH (CTD-PAH), 27 in CTEPH group, and 32 in CTD-non-PAH group were analyzed. Serum haptoglobin levels in PAH group (median 66 mg/dL) were significantly lower than those in CTEPH group (median 94 mg/dL, P = .03) and CTD-non-PAH group (median 79 mg/dL, P = .03). The prevalence of decreased haptoglobin levels was 26% in PAH group, 15% in CTEPH group, and 6% in CTD-non-PAH group. Serum haptoglobin levels had a significant negative correlation (r = −0.66, P < .001) with mean pulmonary artery pressure in PAH group, particularly in CTD-PAH subgroup (r = −0.74, P < .001), but no correlation in IPAH subgroup (r = −0.52, P = .13) and in CTEPH group (r = −0.17, P = .41). Follow-up cases of CTD-PAH showed lowering pulmonary artery pressure led to normalizing serum haptoglobin levels.Serum haptoglobin levels decreased in PAH patients and inversely correlated with pulmonary artery pressure in CTD-PAH patients, suggesting their potential as a surrogate marker for CTD-PAH.