BACKGROUND: To investigate the presence of different isotypes of anti-carbamylated protein (CarP) antibodies in systemic sclerosis (SSc) patients and its association with skin involvement.
METHODS: Sera of 194 SSc patients from the Leiden CCISS cohort, fulfilling ACR/EULAR 2013 criteria and a clinical diagnosis of SSc, 83 patients with other connective tissue diseases/Raynaud\'s Phenomenon, 24 rheumatoid arthritis patients and 98 age and sex-matched healthy controls were tested for the presence of anti-CarP IgG, IgA and IgM, determined by ELISA. Clinical characteristics, that were evaluated in SSc patients, included age, anti-topoisomerase antibodies (ATA), anti-centromere antibodies (ACA) and modified Rodnan Skin Score (mRSS).
RESULTS: The SSc patients were 55 (SD:13) years and 155 (80%) were female. Forty-four (23%) patients tested positive for ATA, and 80 (42%) ACA. The median mRSS was 2 (range: 0; 47). Prevalence of anti-CarP IgG was higher in SSc patients than in healthy controls (8% vs 3%, p = 0.007. Prevalence of anti-CarP IgA and IgM and levels of anti-CarP isotypes were comparable between SSc patients and healthy controls. Fifteen (8%) SSc patients tested positive for anti-CarP IgG, 16 (8%) for anti-CarP IgA, and 36 (19%) for anti-CarP IgM. There were no significant correlations between age and levels of anti-CarP isotypes. No correlation between anti-CarP IgG levels and mRSS was found (r = 0.141, p = 0.049), nor for anti-CarP IgM and IgA levels. Anti-CarP IgA levels were higher in ATA compared to ACA positive SSc patients (ATA: 616 aU/ml [359; 1103]; ACA: 424 aU/ml [300; 673], p = 0.015).
CONCLUSIONS: SSc patients can test positive for Anti-CarP IgG, IgA and IgM. We do not observe a relevant clinical association between anti-CarP antibody response and skin involvement in SSc.

To assess the prognostic significance of skin involvement in breast cancer patients with chest wall recurrence (CWR).
We retrospectively analyzed the clinicopathological data of breast cancer patients with CWR who were diagnosed pathologically between January 2000 and April 2020. Disease-free survival (DFS) was the time from radical resection for CWR to disease recurrence. Progression-free survival (PFS) was defined as the time from the diagnosis of locally unresectable CWR to the first sign of disease progression. Persistent chest wall progression was defined as three consecutive chest wall progressions with no distant organ involvement.
A total of 476 patients with CWR were included in this study. Skin involvement was confirmed in 345 patients. Skin involvement was significantly correlated with a high T stage (p = 0.003), more positive nodes at initial examination (p < 0.001) and lymphovascular invasion (p < 0.001). Kaplan-Meier analysis showed that skin involvement was a predictor of shorter DFS (p < 0.001), including both local disease progression (p < 0.001) and distant disease progression (p = 0.022). Multivariate analysis showed that skin involvement was an independent biomarker for DFS (p = 0.043). Patients with skin involvement were more likely to experience persistent chest wall progression (p = 0.040). After eliminating the potential deviation caused by an insufficient follow-up time, persistent chest wall progression was more likely to be associated with a high N stage (p = 0.002), negative progesterone receptor (PR; p = 0.001) and positive human epidermal growth factor receptor 2 (HER2; p = 0.046) of the primary site, and negative oestrogen receptor (ER; p = 0.027) and PR (p = 0.013) of the chest wall lesion and skin involvement (p = 0.020).
Skin involvement was a predictor of poor disease control in patients with CWR and was closely related to persistent chest wall progression. We stratified the prognosis of individualized treatment for breast cancer patients with CWR to provide new insights into the biological behaviours of the disease.
Skin involvement is a predictor of poor local disease control in breast cancer patients with CWR and a factor contributing to persistent chest wall progression after CWR. We stratified the prognosis of individualized treatment for breast cancer patients with CWR.

Background Healthcare workers (HCWs) were compelled to use personal protective equipment (PPE) during the COVID-19 pandemic to prevent cross-transmission. One of the most significant challenges in responding to the COVID-19 pandemic is the consistent and effective use of PPE to avoid staff exposure and infection. This study aimed to detect and evaluate the adverse effects of PPE and determine the associated risk factors. Methodology This cross-sectional study included 186 randomly selected HCWs at Civil Hospital, Ahmedabad, from May 2022 to July 2022. An anonymous self-administered questionnaire was used for data collection, and data analysis was done using descriptive statistics. Results PPE-related adverse effects were noted among 147 HCWs, with a prevalence of 79.03%. Data analysis showed that factors significantly associated with PPE adverse effects in HCWs were age group 20-40 years (chi-squared (χ2) = 4.119, p = 0.04) and female gender (χ2 = 7.153, p = 0.007). Overall, 30.8% of participants had tested positive while on duty during the pandemic. Similarly, adverse effects were associated with PPE use of more than four hours per day and more than three days per week (χ2 = 5.477, p = 0.02 and χ2 = 6.488, p = 0.01, respectively). The majority of HCWs expressed indentation and pain on the back of the ear (52.7%) and pressure-related injury (39.8%) as adverse effects after wearing masks; skin soaking in sweat (54.83%) due to gloves; profuse sweating due to gown (64.28%); fogging (65.26%) due to googles and face-shield; and discomfort (61.29%). Conclusions The prevalence of adverse effects related to wearing PPE was alarmingly high among HCWs. The major risk factors were age, female sex, and duration of use. Although the majority of healthcare personnel have received vaccinations, the use of PPE has not altered, and severe skin reactions continue to be a global issue with no known solution. To further understand the problem, national data for the impacted healthcare professionals could be helpful. Furthermore, workplace prevention programs are necessary.

Inborn errors of immunity associated with atopy (IEIs-A) are a group of inherited monogenic disorders that occur with immune dysregulation and frequent skin involvement. Several pathways are involved in the pathogenesis of these conditions, including immune system defects, alterations of skin barrier and metabolism perturbations. Current technological improvements and the higher accessibility to genetic testing, recently allowed the identification of novel molecular pathways involved in IEIs-A, also informing on potential tailored therapeutic strategies. Compared to other systemic therapy for skin diseases, biologics have the less toxic and the best tolerated profile in the setting of immune dysregulation. Here, we review IEIs-A with skin involvement focusing on the tailored therapeutic approach according to their pathogenetic mechanism.

UNASSIGNED: Systemic lupus erythematosus (SLE) can significantly influence patients\' quality of life and subjective well-being (SWB), but the relationships between clinical characteristics, SWB, and related psychological factors have been little studied.
UNASSIGNED: To measure SWB in patients with SLE and examine how major clinical determinants, emotional variables, and related positive factors affect SWB.
UNASSIGNED: Overall, 1,110 patients with SLE from the Chinese SLE Treatment and Research Group (CSTAR) and 198 age and gender-matched individuals from the general population without self-reported SLE were invited to complete questionnaires of SWB evaluated by the satisfaction with life scale (SWLS), emotional variables assessed by the patient health questionnaire-9 (PHQ-9), and general anxiety disorder-7 (GAD-7) and related positive factors assessed by the self-esteem scale (SES), general self-efficacy scale (GESE), and Connor-Davidson resilience scale (CD-RISC). The multivariate linear regression was used to examine the relationship between clinical manifestations and SWB.
UNASSIGNED: Life satisfaction was significantly lower (p < 0.001) in patients with SLE than in the general population. Active skin involvement (OR = 0.923, 95% CI = 0.868-0.981, p < 0.05) was negatively associated with life satisfaction scores, and age at enrollment (OR = 1.160, 95% CI = 1.092-1.230, p < 0.001) were positively associated with life satisfaction scores in the multivariate regression model. The cumulative organ damage was significantly associated with depression (OR = 1.085, 95% CI = 1.022-1.153, p < 0.01) and the loss of self-esteem (OR = 1.067, 95% CI = 1.004-1.133, p < 0.05).
UNASSIGNED: SWB provides useful insight into the impact of SLE on psychological health and opportunities to improve quality of life and clinical care.

The skin involvement of myeloid leukaemia is conventionally divided into specific malignant lesions and non-specific benign lesions, and these categories are also applicable in chronic myelomonocytic leukaemia (CMML). According to the 2016 World Health Organization (WHO) classification of tumours of haematopoietic and lymphoid tissues, CMML is defined as a myeloid neoplasm with characteristics of myelodysplastic syndrome (MDS) and myeloproliferative neoplasms (MPNs). As a specific cutaneous sign of extramedullary infiltration, leukaemia cutis (LC) is a rare occurrence in patients with CMML, and only approximately 89 cases have been reported in the literature thus far. The clinical features of LC are varied, and LC in CMML exhibits heterogeneous histopathologic features, with manifestations as cutaneous nodules or papules that are composed of blast cells showing either granulocytic or monocytic differentiation. Skin biopsy and further immunohistochemical examination are essential at the time of diagnosis to evaluate pathological type and determine the clinical course. Generally, once diagnosed as LC in CMML, this unusual skin lesion might be an indicator of transformation to acute myeloid leukaemia (AML) and is associated with a poor prognosis. The main treatment is allogeneic stem cell transplantation (ASCT). Therefore, early diagnosis and accurate identification have important therapeutic and prognostic significance in CMML patients with skin infiltration.

暂无摘要。

Langheransian cell histiocytosis (LCH) is a rare pathology characterized by the proliferation of CD1+ and Langerin+ cells. It can affect all ages, with an estimated prevalence of one to two cases/100,000 habitants. The involvement is often multi-visceral; however, isolated cutaneous involvement can be found in 40% of cases with very variable manifestations. We report the case of 45-year-old women followed for non-insulin-dependent diabetes and primary hyperparathyroidism suffering from isolated and refractory cutaneous histiocytosis.

Mastocytosis is a rare clonal disorder characterized by excessive proliferation and accumulation of mast cells (MC) in various organs and tissues. Cutaneous mastocytosis (CM), the most common form in children, is defined when MC infiltration is limited to the skin. In adults, the most common form is systemic mastocytosis (SM), characterized by MC proliferation and accumulation in organs, such as bone marrow, lymph nodes, liver, and spleen.1 Genetic aberrations, mainly the KIT D816V mutation, play a crucial role in the pathogenesis of mastocytosis, enhancing MC survival and subsequent accumulation in organs and tissues.2,3 CM includes three forms: solitary mastocytoma, maculopapular cutaneous mastocytosis (MPCM), and diffuse cutaneous mastocytosis (DCM). In most children with CM, skin lesions regress spontaneously around puberty; unfortunately, it is not always a self-limiting disease.4 Even if SM occurs occasionally, all children with mastocytosis require planned follow-up over time. Children with mastocytosis often suffer from MC mediator-related symptoms, the most common of which is itching, often triggered by rubbing the lesions. Management of pediatric mastocytosis is mainly based on strict avoidance of triggers. Treatment with H1 and H2 histamine receptor blockers on demand and the availability of epinephrine auto-injectors for the patients to use in case of severe anaphylactic reactions are recommended.

BACKGROUND: Gingivo-buccal complex cancers (GBCC) have an aggressive clinical course in the presence of skin and bone involvement.
OBJECTIVE: This study intends to analyze the clinico-pathological factors affecting local control and survival outcomes in GBCC.
METHODS: This is a retrospective study conducted on 125 GBCC cases from January 2011 to April 2016.
RESULTS: Univariate analysis revealed lymphovascular invasion (LVI) and skin involvement as predictors of poor overall survival (OS) and disease- free survival (DFS). Multivariate analysis showed skin involvement and LVI to be independent prognostic factors towards poor OS. Corresponding results in case of DFS showed skin involvement to be the single most important prognostic factor. With a median follow up of 24 months, the median survival of patients with skin involvement, skin and mandible invasion and isolated mandible invasion, respectively, were 18 months, 12 months and 22 months.
CONCLUSIONS: GBCC with skin involvement portends poor outcome in terms of survival. In the presence of skin invasion, treatment entails liberal soft tissue and bone margin followed by adjuvant treatment.