Clinical assessment of patients with systemic sclerosis: is there a place for thermography?

Recurrent changes of temperature and persistence of cooling along fingers at the room temperature make hands the most frequent region of interest for thermography in systemic sclerosis (SSc). The aim of this study was to evaluate dependance of temperature in hands on a subtype of the disease, immune profile of antinuclear antibodies (ANA), and lung involvement. There were 29 patients with limited cutaneous involvement (lcSSc) and 10 patients with diffuse cutaneous disease (dcSSc) enrolled for the study. To compare measurements to normal values, there were enrolled 29 healthy volunteers (control group). All participants were submitted to thermography with handheld camera FLIR One Pro for iOS, attached to mobile phone iPhone 11, at the fixed temperature of 21 °C. Measurements included average temperature (Tavg) over nailfolds in thumbs and fingers II-V, as well as the difference in average temperatures (TΔ) between metacarpus of the hand and its thumb and fingers II-V. Both thumbs and fingers II-V remained cooler in subjects with dcSSc compared to those with lcSSc. This implicated a significantly greater TΔ along thumbs and fingers II-V in dcSSc group. Although Tavg at nailfolds in SSc patients was not lower than in healthy controls, TΔ remained significantly more pronounced in both lcSSc and dcSSc subjects. A positivity to ACA in lcSSc group was found to be associated with significantly lower Tavg and more pronounced TΔ in fingers II-V than the presence of anti-Scl70 antibodies. Temperature measurements remained statistically independent on a presence of ILD in lcSSc group, but both thumbs and fingers II-V in dcSSc group were warmer in case of lung involvement. The study showed the dcSSc subtype, the positivity of ACA in lcSSc, but not lung involvement were associated with poorer thermal control in the hands of SSc patients. A comparison to healthy controls highlighted the weakness of temperature measurements at nailfolds (Tavg) but increased the value of TΔ in thermography of hands.

The role of proprotein convertase subtilisin/kexin type 9 (PCSK9) in the pathophysiology of psoriasis and systemic lupus erythematosus.

Inflammation and atherogenic dyslipidaemia are often observed in skin diseases and represent an increased risk of cardiovascular disorders. Proprotein convertase subtilisin/kexin type 9 plays an important role in the regulation of serum low-density lipoprotein cholesterol levels. Its biological role, however, seems to go much beyond the regulation of cholesterol metabolism. The article presents potential pathophysiological links between inflammatory process and lipid disorders based on the example of psoriasis and systemic lupus erythematosus.

Proactive therapy: New perspectives for long-term topical treatment of psoriasis.

Mild to moderate psoriasis is most often treated with topical therapy. The article reviews literature on topical treatments that have been assessed in clinical trials and real-life studies lasting at least 12 months. Calcipotriol/bethamethasone dipropionate foam in maintenance treatment following the induction phase can improve efficacy and safety of topical therapy in psoriatic patients. Introduction of new topical nonsteroidal drugs and the wider use of proactive therapy seem to be crucial to achieve satisfactory long-term outcomes in psoriasis.

Does antioxidant-rich diet during pregnancy protect against atopic multimorbidity in children?

BACKGROUND: Genetics and prenatal environmental exposures are indicated in the complex etiopathogenesis and clinical expression of atopic diseases. This study examined the clinical features of infantile-onset atopic dermatitis (AD) in relation to maternal diet during pregnancy. METHODS: Maternal dietary habits were evaluated in terms of the frequency of intake of six different food categories rich in antioxidants or omega-3 fatty acids. RESULTS: One hundred mother-child pairs were recruited, 47 infants (<12 months) and 53 children aged 12-36 months. Forty-six of the children had mild, 41 moderate and 13 severe AD. The other atopic manifestations (alone or associated) included: asthma in 9 cases, allergic rhinitis in 22 cases and food allergy in 33 cases. The presence of asthma in children was significantly associated with a lower level of maternal dietary intake of fruits and vegetables as well as chocolate confectionery, while associations with whole grain breakfast cereals, nuts and seeds, non-alcoholic beverages (coffee, tea, fruit juices) and fish and fish products, were not statistically significant. The age of onset and severity of infantile-onset AD were not linked to any of the food categories considered for analyses. CONCLUSIONS: Healthy diet in pregnant women that is rich especially in antioxidants may provide protection against atopic comorbidities of AD. Further prospective reasearch on the role of maternal diet in primary prevention of atopic diseases is warranted.

Correlation between the infrared thermogram and microvascular abnormalities of the nailfold in patients with systemic sclerosis.

INTRODUCTION: Systemic sclerosis (SSc) is a multisystemic disease with an extensive microvasculopathy. The gold standard for its investigation is nailfold videocapillaroscopy (NVC). AIM: To assess the value of thermography (IRT) for the assessment of microvasculopathy in patients with SSc. MATERIAL AND METHODS: Nineteen patients with limited cutaneous SSc were enrolled in the study. They underwent IRT imaging and NVC. An average temperature (Tavg) at the nailfold and a gradient of temperatures (ΔTavg) between the central metacarpus of the hand and the nailfold was determined for all fingers. NVC pictures were classified to capillaroscopic patterns according to Cutolo et al. system and they were analysed quantitatively to measure the density of capillaries and to calculate capillaroscopic skin ulcers risk index (CSURI) for each finger separately. RESULTS: There was only a moderate correlation (0.4 < r < 0.6) between thermographic parameters and density of capillaries in fingers II-V (r = 0.5; p < 0.001 for Tavg and r = -0.45; p < 0.001 for ΔTavg), but none in thumbs (r = 0.29; p = 0.089 for Tavg and r = -0.19; p = 0.275 for ΔTavg). Early pattern was associated with a significantly greater surface temperature (Tavg) of nailfolds and essentially milder ΔTavg in fingers II-V when compared to all other capillaroscopic patterns in fingers II-V. Surface temperature (Tavg) was significantly lower and ΔTavg was markedly more pronounced in fingers II-V with a greater risk of development of digital ulcers (DU) calculated by CSURI. CONCLUSIONS: Although IRT measurements correlate only moderately with density of capillaries, this technique seems to be substantial to determine the capillaroscopic pattern and to identify patients at greater risk of DU development.

Narrow-Band Reflectance Spectrophotometry for the Assessment of Erythematous and Hyperpigmented Skin Lesions in Localized Scleroderma: A Preliminary Study.

BACKGROUND: Localized scleroderma (LoS) is an inflammatory fibrosing disease of the connective tissue, whose esthetic sequelae are atrophic skin lesions with hyperpigmentation. The key element of diagnostic and therapeutic procedures is the assessment of the disease's severity and damage. The study objective was to analyze the usefulness of narrow-band reflectance spectrophotometry (NBRS) to assess erythema and hyperpigmentation in LoS lesions. MATERIALS AND METHODS: Erythema indexes (EI) and melanin indexes (MI) were determined with the use of DermaLab Combo skin colour probe for LoS lesions and symmetrically located areas of normal skin. Then, relative percentage differences were determined for EI and MI, which were compared with the visual assessments of erythema and hyperpigmentation according to the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT). RESULTS: A total of 84 LoS lesions were studied in 41 patients. The study showed a moderate correlations between the spectrophotometric measurements and clinical assessments of erythema as well as hyperpigmentation (Spearman correlation coefficient, r), r = 0.37; p = 0.00047 and r = 0.55; p=0.0000001, respectively. CONCLUSION: NBRS seems to be a useful tool to assess the severity of erythema and hyperpigmentation in LoS lesions. Further studies are required in order to compare the spectrophotometric results with other objective methods.

Frontal fibrosing alopecia: a disease that remains enigmatic.

Frontal fibrosing alopecia (FFA) is recognized to represent a generalized process of inflammatory scarring alopecia. Apart from the classic form affecting the frontal hairline, there are a range of disease manifestations involving loss of eyebrows and of eyelashes, loss of peripheral body hair, fibrosing alopecia in a pattern distribution, facial and extrafacial skin, mucous membrane, and nail involvement. Classic linear, diffuse "zigzag", pseudo "fringe sign", androgenetic alopecia-like, cockade-like, ophiasis-like and incomplete patterns are distinguished. The aetiology of FFA remains obscure, but a number of pathogenetic hypotheses and treatments to halt disease progression have been proposed.

Does maternal pet ownership during pregnancy influence severity of child's atopic dermatitis?

This study investigated associations between atopic dermatitis (AD) and selected prenatal and perinatal factors. Maternal exposure to pets during pregnancy was associated significantly with lower severity of child's AD (P = 0.045). A trend towards significance of relation was demonstrated between AD severity and place of residence and maternal exposure to stress.

Health-related quality of life and its influencing factors in adult patients with localized scleroderma - a cross-sectional study.

BACKGROUND: Localized Scleroderma (LoS) is an autoimmune connective tissue disease that affects skin and less commonly subcutaneous tissues. The illness occurs in children and adults, and may have a serious impact on health-related quality of life (HRQoL). The goal of this study was to explore what factors might deteriorate scores on HRQoL measures in adult LoS patients. METHODS: Detailed information on the demographic and clinical features of LoS patients was collected. The HRQoL was assessed using Skindex-29 and Short form 36 (SF-36) questionnaires. RESULTS: Thirty three women and seven men with LoS were enrolled. Female gender negatively influenced scores for the emotion subscale of Skindex-29. Multiple-factor linear regression analysis confirmed, as with single-factor analysis, that the causes of low SF-36 physical component score (PCS) were subjective symptoms (pruritus, pain, paresthesia), musculoskeletal manifestations and older age at the time of survey, while rural area of residence negatively affected the SF-36 mental component score. Additionally, single-factor analysis showed that the SF-36 PCS was related to the LoS cutaneous assessment tool (LoSCAT) summary score. CONCLUSIONS: Apart from a clinical presentations, sociodemographic characteristics, including older age, female gender and living conditions, may impair HRQoL of LoS patients. Further studies that will examine the role of these factors for physical and mental functioning of adults with LoS are needed.

Associations between Disease Activity/Severity and Damage and Health-Related Quality of Life in Adult Patients with Localized Scleroderma-A Comparison of LoSCAT and Visual Analogue Scales.

Localized scleroderma (LoS) is a chronic fibrosing disorder of the skin and, less commonly, subcutaneous tissues. As the disease causes subjective symptoms, cosmetic defects, and, at times, functional disability, subjects with LoS experience deterioration of their health-related quality of life (HRQoL). The influence of disease activity/severity and damage status on HRQoL measures in patients with LoS is scarcely known. Physician-reported measures (modified LoS skin severity index, LoS skin damage index, physician global assessments of the disease activity/severity and damage) and patient-derived measures (patient global assessments of the disease activity/severity and damage) were obtained in adult LoS patients. Their HRQoL was measured with Skindex-29 and Short Form-36. The patients' assessments of disease activity/severity and damage in LoS differed from the assessments by the physicians. The patients' predominant concerns centered on LoS-related damage, whereas the physicians' concerns focused on features of disease activity. Visual analogue scales bore some relation to the HRQoL, and they seem to be important in a holistic approach to the patient and should not be omitted in LoS evaluation.

Recent advances in the workup and management of Raynaud phenomenon.

Raynaud phenomenon (RP) is defined as recurrent, reversible episodes of vasospasm involving peripheral small vessels, typically in the fingers and toes. Primary (idiopathic) RP is common (it occurrs in about 5% of the general population) and is usually benign. Secondary RP accounts for 10% to 20% of all RP cases and may be associated with complications such as tissue loss, ulcers, and gangrene. Systemic sclerosis (SSc) or, more rarely, other connective tissue diseases are the main underlying conditions. A detailed clinical history and careful physical examination may be helpful in identifying the cause. Routine investigations include a full blood count, measurement of erythrocyte sedimentation rate, C­reactive protein, antinuclear antibody levels, biochemical profile, thyroid function tests, protein electrophoresis, chest X­ray, and nailfold capillaroscopy. Capillaroscopy can facilitate a very early diagnosis of SSc. Doppler ultrasound is recommended to evaluate the risk of pathologies in large to medium-sized arteries. Lifestyle modifications may be sufficient to control primary RP, but some patients, and most with secondary RP, require pharmacologic treatment. Several medications are proposed to manage RP and its complications, such as calcium channel blockers, phosphodiesterase type 5 inhibitors, intravenous prostanoids, and topical nitrates. However, scientific evidence for the use of these drugs is still weak to moderate. Despite the lack of efficacy of bosentan in RP treatment, this medication is approved for the secondary prevention of digital ulcers in patients with SSc. In conclusion, the management of RP still represents a challenge. Collaboration between healthcare professionals, patient organizations, and the society could encourage earlier medical assessment of people at risk of SSc.

An extended high-frequency ultrasound protocol for assessing and quantifying of inflammation and fibrosis in localized scleroderma.

BACKGROUND: Clinical characteristics of the lesions are used to identify activity and damage in localized scleroderma (LoS). For high-frequency ultrasound (HF-US), the features of active lesions were described. MATERIALS AND METHODS: Clinical signs of activity and damage in LoS lesions were assessed with the use of Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and HF-US by two examiners independently. All US images were obtained using a 20 MHz HF-US (DermaLab System, Cortex Technology, Hadsund, Denmark). The dermal thickness (DT) and echogenicity (intensity score, IS) of the LoS lesional dermis were measured in the area of each lesion with the highest score for erythema (ER), skin thickness (ST), and dermal atrophy (DAT). Measurements were compared to the site-matched unaffected skin. The relative difference of DT and IS values was calculated between each lesion and its normal control for comparison among different clinical scores for ER, ST, and DAT. RESULTS: A total of 92 lesions in 40 adult patients were examined with HF-US. Thirty one lesions were erythematous, 26 were in sclerosis, and 35 were in atrophy. A correlation between the clinical evaluation of the LoS lesions and US measurements was found. The sensitivity and specificity of HF-US were 97% and 90%, respectively. The positive predictive value was 83%, negative predictive value-98%. Interrater reliability was excellent for LoSCAT and HF-US findings. CONCLUSION: High-frequency ultrasound allows an accurate assessment of the inflammatory and fibrotic skin lesions in LoS.

Facts and speculations on vitamin d in prevention and treatment of atopic dermatitis

Recently, age- and weight-specific recommendations for prevention, supplementation and treatment of vitamin D (VD) deficiency in the general population and in groups at increased risk of the deficiency (i.a. atopic dermatitis) has been presented by the Polish Experts group. VD regulates many physiological processes in the skin ranging from cellular proliferation, differentiation, and apoptosis to barrier maintenance and immune functions. VD deficiency has been associated with increased incidence and severity of atopic dermatitis. In addition, VD supplementation was shown to decrease atopic dermatitis severity in several studies, but the findings obtained in systematic reviews and meta-analyses are controversial.

Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) adapted for use in adult patients: report from an initial validation study.

BACKGROUND: Localized scleroderma (LoS) affects both children and adults and is associated with permanent functional and cosmetic impairment, and reduced quality of life predominating in adults. The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) is a clinical instrument designed to measure an activity and damage of LoS. It has been validated for use with pediatric LoS patients. This study assessed the validity and reliability of the LoSCAT adapted for use in adults. METHODS: Before the initiation of the study two examiners participated in an intensive training course carried out by an expert in LoS. Appendices describing each LoSCAT domain were prepared. Features determining disease activity and damage in adult LoS patients were identified to properly evaluate the physician (Phys) and patient (Pt) global assessment (GA) of disease activity (A)/severity (S) and damage (D), which were used to assess convergent validity of the LoSCAT. Correlations of physician- and patient-derivied measures with Skindex-29 were also analysed. RESULTS: The study included 40 adult LoS patients (33 females and 7 males) with different subtypes of LoS. Intra and inter-rater reliability of the LoSCAT was found to be excellent. Positive correlations were observed between the PhysGA-A, PhysGA-D, PtGA-A and the LoSCAT's domains, while no correlations between them and the PtGA-D were found. There were no relationships between LoSCAT's components and Skindex-29. CONCLUSIONS: Despite the LoSCAT is a reliable tool for an assessment of cutaneous lesions, additional health status instruments are necessary to a holistic approach to LoS in adults.

High acute phase protein levels correlate with pulmonary and skin involvement in patients with diffuse systemic sclerosis.

Objective This study was performed to evaluate the serum amyloid A (SAA) and C-reactive protein (CRP) levels in patients with diffuse systemic sclerosis (dSSc) in relation to a control group, disease duration, and skin and pulmonary involvement. Methods This case-control study included 18 patients with early dSSc, 15 patients with late dSSc, and 15 healthy controls. The SAA and CRP levels, modified Rodnan skin score (mRSS), and diffusing capacity of the lungs for carbon monoxide (DLCO) were determined in all patients. Results The SAA and CRP levels were significantly higher in patients with early and late dSSc than in healthy controls. The frequency of detection of elevated SAA and CRP levels was approximately 66% and 85%, respectively. A significant correlation was found between the SAA and CRP levels in patients with dSSc. The SAA and CRP levels were inversely correlated with DLCO. The CRP level was positively correlated with the mRSS. Conclusions High SAA and CRP levels could serve as biomarkers for pulmonary involvement. The serum CRP level accurately reflects the extension of skin thickening in patients with dSSc.

Thermal imaging and dermoscopy for detecting inflammation in frontal fibrosing alopecia.

BACKGROUND: Frontal fibrosing alopecia (FFA) is an inflammatory condition of the scalp, which leads to scarring and slowly progressive recession of frontotemporal and/or frontoparietal hairline. Choice of FFA treatment is highly dependent on accurate assessment of disease phase, as medical treatments are effective only during the initial inflammatory stage. METHODS: To objectively quantify the activity of the inflammatory process in FFA, 22 female patients were examined by both infrared thermography and optical dermoscopy before tissue sampling. The presence of perifollicular erythema or scaling was considered to be strongly suggestive for appropriate scalp biopsy site. Skin temperature differences in dermoscopy-selected area >0.5°C for temperature of the reference area (lower area of forehead) were considered abnormal. For evaluation of the inflammatory infiltrate, a grading scale was used. RESULTS: The scale classified 14 (64%) subjects as being in the active disease phase of FFA and eight (36%) as in the inactive one. Using the thermography findings, 17 (77%) patients were considered to have the active FFA and five (23%) patients had the inactive one. The clinical assessment of active FFA was most accurate using thermal imaging combined with dermoscopy, with a sensitivity of 64%, a specificity of 88%, a positive predictive value of 90%, and an negative predictive value of 58%. CONCLUSION: We believe that infrared thermography is a noninvasive and accessible imaging modality that may serve as a complementary tool in FFA diagnosis.

Transcriptional Activity of Nuclear Factor κB Family Genes in Patients with Systemic Sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is a connective tissue disease of unknown etiology and unclear pathogenesis. Evaluation of the activation of nuclear factor κB (NF-κB) family genes IκBα, p50, p52, p65, and c-Rel, potentially involved in the regulation of immunity, inflammation, angiogenesis, and tissue remodeling in SSc, was carried out. METHOD: The study included 19 patients with limited SSc, 11 patients with early SSc, and 10 healthy persons constituting the control group. Real-time QRT-PCR was used to evaluate the mRNAs in peripheral blood samples. RESULTS: The patients with early SSc showed a decrease in transcriptional activity of IκBα inhibitor and c-Rel subunit. Transcriptional activity decrease in the other patients with limited SSc included genes encoding c-Rel and p50, subunits of NF-κB factor. CONCLUSIONS: Deregulation of intracellular signal transduction by NF-κB takes place at the beginning of SSc and in its fibrosis stage. Associations between clinical variables and NF-κB related gene expression as well as the activation of NF-κB family members in SSc patients should be addressed in future studies.

Neurological abnormalities in localized scleroderma of the face and head: a case series study for evaluation of imaging findings and clinical course.

INTRODUCTION: Localized scleroderma (LoS) of the face and head is often associated with neurological manifestations and/or imaging abnormalities in the central nervous system (CNS). CASE SERIES: We present an analysis of 20 cases of LoS affecting the face and head. The CNS symptoms and/or abnormalities in high-resolution computed tomography (HRCT) and/or magnetic resonance imaging (MRI) were observed in 12 patients (60%). In addition to the mild and unspecific disorders (e.g. headaches), serious neurological complications probably in the course of vasculitis were revealed: epilepsy (in two patients), epilepsy and pyramidal sings (in one patient). Neurological disorders and LoS occurred at the same time (in three patients) or at the course of the disease (nine patients) and no later than 29 years since the onset of the disease. No link between neurological disorders and the LoS clinical morphology, immunological and other laboratory parameters has been established. CONCLUSIONS: CNS involvement is not correlated with the clinical course of the facial and head LoS and may occur years after the disease initial symptomatology. Imaging follow-up is not required if there is not any emerging neurological symptom. In some cases, however, both HRCT and MRI are useful for monitoring disease evolution and addressing therapeutic choices.

Dietary strategies for primary prevention of atopic diseases - what do we know?

The paper refers to the recently published empirical data and systematic reviews on the impact of diets, foods, nutrients and bioactive substance exposures in pregnancy and in early infancy, on the development of atopic disorders. The results of studies referring to a broad range on dietary factors are mostly conflicting. There are several limitations of these researches. Based on the existing information, it is not possible to establish the role of antioxidants and vitamin D supplementation in atopic disease development. There is no evidence of major effects of prenatal use of folic acid on asthma or allergies. The association of some nutritional interventions with less atopic sensitization seems rather speculative even if such an effect has not been found for some other foods. The findings indicate rather a balanced and diverse diet without restrictions than a special dietary protocol. Farming-related exposures may protect against the development of atopic disorders in children. The hypothesis that the early introduction of complementary food, including the potentially allergenic foods, may reduce the risk of food allergy and atopic dermatitis is currently tested. Long-chain polyunsaturated fatty acids and probiotics seem to be promising candidates for allergy prevention. But specific recommendations regarding pre- and postnatal supplementation strategies, dose, treatment duration etc., are still undetermined. Longitudinal intervention studies in cohorts of pregnant women or newborn infants are needed to match the proper strategies in these issues.