Search

Background: Regulatory T cells (Tregs) play an important role in autoimmune diseases. In skin, the presence of Tregs is thought to be mandatory for suppression of autoreactive T cells. Here, we assess the number of Tregs in skin of healthy subjects and patients with an autoimmune dermatosis., Methods: Immunohistochemical stainings for CD3 and FOXP3 on skin biopsies of healthy subjects and subjects with psoriasis, vitiligo, pemphigus vulgaris, bullous pemphigoid, and halo nevus to assess the number of T and regulatory T cells, respectively., Results: Low numbers of CD3+ and FOXP3+ cells were seen in the skin of healthy controls (median = 0.5%). A significantly higher frequency of Tregs was seen in lesional skin of patients with psoriasis (median = 12.4%) and patients with bullous pemphigoid (median = 10.1%) as compared to controls. In vitiligo (median = 0.0%), pemphigus vulgaris (median = 5.2%), and halo nevi (median = 5.4%), no significant difference in number of FOXP3+ cells was observed when compared to controls., Conclusions: As confirmed in the literature, few Tregs were seen in healthy skin. A high number of Tregs were present in lesional skin from patients with psoriasis and bullous pemphigoid. These results support the hypothesis that not a decrease in number but rather a decrease in function of Tregs would be at the basis of autoimmune skin diseases, which could result in unrestrained activation autoreactive T cells in skin of patients with autoimmune dermatoses., (© 2013 The International Society of Dermatology.)

Lichen sclerosus is a relatively common chronic inflammatory skin disease that predominantly affects the anogenital area. Accumulating evidence indicates that lichen sclerosus in women may be associated with other autoimmune disease, whereas this association seems to lack in male patients. We retrospectively evaluated the prevalence of autoimmune diseases and serological parameters indicative for autoimmunity in male and female patients with lichen sclerosus. Of the 532 patients (396 women, 136 men; 500 adults, 32 children; mean age: 49 years; range 1-89 years; female:male ratio 3:1), 452 (85%) had genital and 80 (15%) had extragenital disease. In women, lichen sclerosus was significantly more often associated with at least one autoimmune disease as compared to men (odds ratio [OR] 4.3, 95% confidence interval [CI] 1.9-9.6; p<0.0001). Moreover, female patients with lichen sclerosus had sinificantly more often associated autoimmune thyroid diseases (OR 4.7, 95% CI 1.8-11.9; p<0.0002), antithyroid-antibodies (OR 2.7, 95% CI 1.1-6.5; p=0.023), and elevated autoantibodies (OR 4.1, 95% CI 1.9-9.3; p<0.0001) as compared to male patients. This observation is suggestive for a different pathogenetic background in male and female patients.

Amicrobial pustulosis of the folds (APF) is characterized by relapsing, primary aseptic, pustular eruptions mainly affecting the cutaneous folds, scalp, and periorificial regions such as the mouth, external ear canal, and nostrils. APF is a rare condition that has previously been reported in 25 women exhibiting a wide spectrum of autoimmune abnormalities, particularly systemic lupus erythematosus. Histology of the skin lesions usually shows spongiform pustulation in the upper layer of the epidermis and a polymorphonuclear infiltrate in the dermis. APF must be differentiated from other noninfectious pustular diseases such as subcorneal pustulosis (Sneddon-Wilkinson disease) and pustular forms of psoriasis. APF should be included in the spectrum of reactive neutrophilic dermatoses, even though the exact pathomechanisms remain obscure. However, the striking female predominance may be of pathogenetic significance. Based on the small number of previously reported patients, the most effective therapy seems to be medium-dose systemic corticosteroids.

Background: Laboratory-based biomarkers accurately presenting systemic lupus erythematosus (SLE) disease activity may have a practical value in clinical routine. As shown in many other conditions, complete blood count (CBC)-derived biomarkers may also play a role in SLE. Objectives: We aimed to study for the first time the pan-immune-inflammation value (PIV, monocytes x platelets x neutrophils/lymphocytes) and the more established systemic immune-inflammation index (SII, neutrophils x platelets /lymphocytes) in SLE patients and correlate it with serological and clinical findings including disease outcomes. Methods: In this retrospective multicentric investigation, we reviewed the clinical records of 148 SLE who had an available CBC at baseline. The latter served for the determination of the neutrophil-to-lymphocyte ratio (NLR), SII, and the PIV. Control groups were studied as well. Univariable as well as multivariable statistics were employed. Results: The values for baseline systemic immune-inflammation biomarkers (SIIB) studied were significantly (p < 0.0001) higher than those observed in healthy controls but comparable to those obtained from patients with other inflammatory conditions. Multivariable analysis revealed that ANA titer > 1:640 remained the only significant (p < 0.0001) baseline predictor of SLE flare (odds ratio: 7.6, 95% CI 3.1 to 18.8). Improvement of SLE following treatment was associated with the absence of lymphopenia as well as ANA > 1:640 (p = 0.041). The SLEDAI-2K significantly correlated with NLR, SII, CRP, lymphocytes, and monocytes only on univariable testing. Conclusions: Compared to healthy controls the CBC-based SIIB investigated are significantly increased in SLE patients. However, SIIB do not appear to be useful in managing SLE clinically. Nevertheless, we confirm that higher ANA titers can predict flares of SLE. [ABSTRACT FROM AUTHOR]

This article discusses the first European guideline for paraneoplastic autoimmune multiorgan syndrome (PAMS), also known as paraneoplastic pemphigus. The guideline aims to improve the diagnosis and treatment of this rare and often fatal condition. However, the authors express concerns about the guideline's neglect of the diagnostic significance of autoreactive T lymphocytes and the omission of bronchiolitis obliterans (BO) as a clinical diagnostic criterion. They argue that BO is a common and life-threatening complication of PAMS and should be included in the diagnostic criteria. The authors also highlight the role of cytotoxic lymphocytes in the pathogenesis of PAMS and suggest that autoreactive T-cell responses may dominate in some cases. They propose that BO should be considered a diagnostic criterion along with other clinical criteria and lesional histopathology. [Extracted from the article]

Systemic sclerosis (scleroderma) (SSc) is a rare autoimmune disorder characterized by excessive production of collagen. Extracorporeal photopheresis (photochemotherapy, phototherapy) (ECP) involves repeated exposure of peripheral blood lymphocytes to ultraviolet A (UVA) radiation. The rationale for treating patients with SSc by ECP lies in its presumed immunomodulatory effects, though, rigorous data on the specific effects of ECP are limited, particularly in patients with SSc. The objective was to evaluate the effects of extracorporeal photopheresis as a treatment modality for patients with SSc. We searched the databases CENTRAL and MEDLINE on 13 March 2022 and included randomized clinical trials (RCTs) on patients diagnosed with SSc and treated with ECP. Primary outcome was the change of skin scores. We applied independent extraction and judgment by multiple observers. We conducted a meta‐analysis applying the inverse variance method and the random effects model; the main outcome measure was standard mean difference of skin scores. We identified three relevant RCTs including 162 randomized (132 analyzed) people who received ECP in a simple parallel design. Pooled data of the three studies were indifferent. We estimated a standard mean difference from baseline of −0.11 (95% confidence interval −0.45 to 0.23), p = 0.54, I2 = 0%. We did not identify serious treatment‐related adverse events. The evidence base for extracorporeal photopheresis on skin scores in patients with systemic sclerosis was not high enough to support a superior effect when compared to no treatment, sham photopheresis, or D‐penicillamine. [ABSTRACT FROM AUTHOR]

Paraneoplastic autoimmune multiorgan syndrome (PAMS) is a life-threatening autoimmune disease associated with malignancies. Here, we present a patient initially misdiagnosed with "chronic" Stevens–Johnson syndrome. Over a year later, the patient was diagnosed with stage IV follicular lymphoma and treated with an anti-CD20 antibody. At this time, his skin condition had significantly worsened, with erythroderma and massive mucosal involvement, including in the mouth, nose, eyes, and genital region. Histopathology revealed lichenoid infiltrates with interface dermatitis, dyskeratoses, necrotic keratinocytes, and a dense CD8+ infiltrate with strong epidermotropism. Direct and indirect immunofluorescence tests for autoantibodies were negative. Remarkably, we retrospectively discovered a chronic increase in peripheral CD8+ lymphocytes, persisting for over a year. Consequently, the patient was diagnosed with antibody-negative PAMS. Three weeks later, he succumbed to respiratory failure. This dramatic case highlights the challenges in diagnosing PAMS, particularly in cases where immunofluorescence assays are negative. Importantly, we observed, for the first time, a chronic excess of CD8+ peripheral blood lymphocytes, associated with PAMS, consistent with the systemic, autoreactive T-cell-driven processes that characterize this condition. [ABSTRACT FROM AUTHOR]

We report the case of a 38-year-old Caucasian female presenting asymptomatic plaques of fine wrinkling and perifollicular papular protrusions especially on the trunk. Histological examination evidenced loss of elastic fibers in the mid-dermis due to elastophagocytosis. with giant cells and granuloma formation. Moreover, elevated titers of thyroid autoantibodies were detected and thyroid ultrasound revealed echo-poor tissue. These findings met the diagnoses of mid-dermal elastolysis and Hashimoto's thyroiditis. This association has nor been reported before. We present a comprehensive overview of the literature and discuss the pathogenetic aspects of mid-dermal elastolysis and the significance of the association with Hashimoto's thyroiditis. [ABSTRACT FROM AUTHOR]