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This article discusses a case of cryoglobulinemic vasculitis complicating Sjögren's syndrome with IgA accumulation. The patient, a 90-year-old Japanese female, presented with bilateral lower limb purpura and joint pain. Laboratory investigations showed elevated levels of various markers, including immunoglobulin G (IgG), immunoglobulin A (IgA), and rheumatoid factor (RF). The patient was diagnosed with primary Sjögren's syndrome complicated by cryoglobulinemic vasculitis. Treatment with prednisolone and azathioprine improved the symptoms. The article highlights the importance of careful examination to identify underlying conditions such as Sjögren's syndrome or cryoglobulinemia in cases clinically diagnosed as IgA vasculitis. [Extracted from the article]

The association between immunoregulatory cytokines, such as interleukin (IL)‐10 or IL‐35, and dipeptidyl peptidase‐4 inhibitor (DPP4i)‐related bullous pemphigoid (BP) has not been evaluated. Serum IL‐10 and IL‐35 levels were measured in 39 patients with BP (24 males and 15 females; 6 DPP4i‐related and 33 DPP4i‐unrelated BP patients) and 10 healthy controls. The number of CD26+ cells in the dermis around bulla on sections was counted immunohistochemically for 12 patients (six patients with DPP4i‐related BP and six randomly sampled patients with DPP4i‐unrelated BP). Patients with DPP4i‐related BP had lower levels of serum eosinophils (DPP4i‐related vs. DPP4i‐unrelated BP: 476.1 ± 234.0 vs. 911.3 ± 948.8/μL; p = 0.537) and a higher rate of infiltrating CD26+ cells (32.9 ± 7.1% vs. 15.7 ± 4.4%; p = 0.01). There were no significant differences in serum IL‐10 (6.77 ± 0.24 vs. 6.84 ± 0.20 pg/mL), serum IL‐35 (2.63 ± 0.17 vs. 2.63 ± 0.21 pg/mL), serum anti‐BP180NC16a antibodies (67.31 ± 37.4 vs. 76.18 ± 54.59 U/mL) and Bullous Pemphigoid Disease Area Index before treatment in this study. Serum IL‐10 and IL‐35 levels do not increase in patients with BP and may not be a candidate for a therapeutic target for BP. An increase in CD26+ cells might be associated with DPP4i‐related BP. [ABSTRACT FROM AUTHOR]

Patients with SSc often have anti-mitochondrial antibodies, as in our case.[4] Primary biliary cholangitis was excluded by hematological findings and a liver biopsy. We should think about the rare possibility that anti-mitochondrial antibodies occurred in her myositis.[7] Although the degree of serum CK was compatible, she did not have cardiac involvement, fibrosis or granulomatous change in the deltoid muscle biopsy. Limited cutaneous systemic sclerosis with myositis showing drug-induced liver injury and thrombotic microangiopathy. [Extracted from the article]

Objective: For patients with connective tissue disease‐associated interstitial lung disease (CTD‐ILD), early medical intervention would be desirable. This study analyzed the real‐world, single‐center use of nintedanib for CTD‐ILD patients. Methods: Patients with CTD who received nintedanib from January 2020 to July 2022 were enrolled. Medical records review and stratified analyses of the collected data were conducted. Results: Reduction in the percentage of predicted forced vital capacity (%FVC) was seen in the elderly group (>70 years; P =.210), males (P =.027), the late group who started nintedanib >80 months after confirmation of an ILD disease activity (P =.03), the severe %DLco (diffusing capacity for carbon monoxide as a percentage of predicted) group (<40%; P =.20), the group who had extensive pulmonary fibrosis at the beginning of nintedanib (pulmonary fibrosis score >35%), and the low‐dose group (nintedanib 50‐100 mg/d; P =.40). %FVC did not decrease by >5% in the young group (<55 years), the early group who started nintedanib within 10 months after confirmation of an ILD disease activity, and the group whose pulmonary fibrosis score at the beginning of nintedanib was <35%. Conclusion: It is important to diagnose ILD early and start antifibrotic drugs with proper timing for cases in need. It is better to start nintedanib early, especially for patients at risk (>70 years old, male, <40% DLco, and >35% areas of pulmonary fibrosis). [ABSTRACT FROM AUTHOR]

In this study, dermoscopy was applied to determine the site of skin biopsy in cases of palmoplantar pustulosis (PPP) with invisible tiny pustules or vesicles. Fifty‐two clinically diagnosed PPP patients (11 men and 41 women) were observed by a dermoscope and underwent skin biopsy. Nineteen patients had visible pustules, whereas 33 had no visible pustules. Pustules were detected by a dermoscope on palmoplantar lesions in 17 of the 33 PPP patients. In contrast, 16 patients showed no presence of pustules or vesicles observed by a dermoscope. In this group, biopsy specimens were taken from scaly lesions. Out of the 16 patients, 10 histologically showed pustules in the epidermis. We finally detected pustules for 46 patients among 52 who were clinically diagnosed with PPP. As a result, we detected pustules in PPP for 19 patients on "inspection", and 36 patients on "inspection and dermoscopy", and 46 patients on "inspection, dermoscopy, and biopsy". Dermoscopy is significantly useful comparing the detection rate (inspection, 0.41; inspection and dermoscopy, 0.78; p = 0.0003; using χ2‐test). In addition, we also should learn from the results that the detection rate goes down to 0.78 without a biopsy. Thus, dermoscopy is certainly a useful tool for invisible pustules and also undetectable pustules to detect pustules in PPP. Moreover, we objectively showed that "inspection, dermoscopy, and biopsy" are the best way to make a PPP diagnosis. [ABSTRACT FROM AUTHOR]

Trimethoprim–sulfamethoxazole (TMP/SMX) combination is used for the prophylaxis of Pneumocystis pneumonia (PCP). Although TMP/SMX is frequently used in dermatology for cases treated with corticosteroids and/or immunosuppressants, it is often difficult to continue the administration of TMP/SMX due to adverse events. There are only a few reported studies on the prophylaxis of PCP in dermatology. This is the first review focused on adverse events of TMP/SMX among patients with dermatological diseases compared with previous reports. In this study, we retrospectively investigated 132 cases treated with TMP/SMX and examined the adverse events. Adverse events occurred in 32 cases (24.2%) and the incidence in this study was higher than in previous reports. Thrombocytopenia occurred in 17 cases (12.5%), which was the most frequent adverse event. The possible causes of adverse events were that the standard dose of TMP/SMX may be excessive for most Japanese, in addition to the long administration period, and the concomitant use of corticosteroids and/or immunosuppressants in almost all cases. We must consider the risks of PCP and adverse events of TMP/SMX in each case. It is desirable to examine possible administration methods that can be continued by adjusting the dose and interval of TMP/SMX. [ABSTRACT FROM AUTHOR]