Morphea Patients Research Articles

Exploring the relationship between morphea and malignancy: a decade-long single-center study of 204 patients

The association between systemic scleroderma and malignancy is well-documented, but there is limited data on the relationship between morphea and malignancy. This study aims to assess the incidence and types of malignancies in morphea patients, comparing demographics, clinical characteristics, treatments, and outcomes between those with and without malignancy. We conducted a retrospective study of 204 morphea patients treated at Rabin Medical Center between 2012 and 2023. Data on demographics, clinical subtypes, comorbidities, treatments, and outcomes were collected. Patients were categorized based on malignancy status and the timing of malignancy relative to their morphea diagnosis. Among the 204 patients (154 women and 50 men, mean age 53.7 ± 20 years), 47 (23%) developed malignancies. In 29 patients (61.7%), malignancy occurred before the onset of morphea; in 23 patients (48.9%), it occurred after morphea. Five patients (10.6%) had malignancies both before and after the diagnosis of morphea. Patients with malignancy were significantly older than those without (64.7 ± 15.1 years vs. 50.3 ± 20 years, p < 0.0001). The all-cause mortality rate was higher in the malignancy group compared to those without malignancy (23.4% vs. 3.8%, p = 0.00002). Moreover, mortality was higher in patients whose malignancy occurred after morphea than in those whose malignancy preceded morphea (26% vs. 17.2%). The most common post-morphea malignancies in our cohort included non-melanoma skin cancer, cervical cancer, breast cancer, stomach cancer, and lung cancer. The most common pre-morphea malignancies included breast cancer, non-melanoma skin cancer, colon cancer, prostate cancer, and testicular cancer. This study suggests potential associations between morphea and malignancies, influenced by patient age, sequence of diagnosis, and treatment regimens. Further control studies are needed to explore these relationships more definitively.

The Serological Evidence of Positive Rheumatoid Factor in Morphea Patients and Its Relation to Disease Severity.

Background and objective Morphea, or localized scleroderma (LS), is an autoimmune skin disorder characterized by inflammation and sclerosis. Its potential causes include infections, genetic predisposition, and trauma. The disease involves cycles of inflammation and fibrosis, leading to skin hardening and scarring, which can cause deformities ifuntreated. Researchexploring the link between morphea and rheumatoid factor (RF), a marker associated with other autoimmune conditions, is ongoing. This study aimed to examine the less-explored role of RF, a marker typically linked to rheumatoid arthritis (RA), in the severityof morphea. It focused on assessing the levels of RFamong morphea patients and its correlation with disease severity, intending to provide deeper insights intothe condition and its management. Methods This study involved a simple randomized cross-sectional analysis to evaluate the role of the RF in measuring morphea severity among patients at the Dermatology and Venereology Department of Al-Sader Teaching Hospitalfrom October 2022 to December 2023. We included participants with clinically and laboratory-confirmed morphea while excluding those with other autoimmune dermatological diseases, recent systemic steroid or immunosuppressive therapy, and pregnant women. The assessment of disease severity was done by utilizing the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT).Statistical analyses were performed using SPSS Statistics version 27 (IBM Corp., Armonk, NY), with a significance threshold of p<0.05. Results Elevated RF levels were significantly associated with increased morphea severity, with severe cases showinghigher RF levels (mean: 30.34 U/mL) compared to moderate (25.83 U/mL) and mild cases (21.56 U/mL) (p = 0.028). However, no significant correlation was found between RF levels and demographic factors such as age, gender, or occupation. Patients with high RF levels had a longer disease duration (mean: 57.15 years) compared to those with normal levels (25.83 years, p = 0.020). Significant differences were observed in lesion distribution on the back (p = 0.002). Logistic regression indicated that severe morphea patients were more likely to have elevated RF levels [odds ratio (OR): 1.158, p = 0.014]. Conclusions This study enriches our understanding of RF's role in morphea, revealing no significant correlation with demographic factors but suggesting its potential role in disease chronicity and severity.

A Comparison of Clinical, Demographic and Treatment Characteristics of Pediatric-Onset and Adult-Onset Patients Diagnosed With Localized Scleroderma.

Morphea localized scleroderma (LS) is a rare skin disease with unknown pathogenesis, which causes sclerosis of the dermis and subcutaneous tissue. It was aimed to compare the characteristics of patients with pediatric and adult-onset morphea. A retrospective analysis was performed on the records of 183 adult morphea patients. The demographics, clinical and laboratory characteristics, and treatment options of the patients were recorded. Adult patients with morphea over the age of 18 were divided into two groups according to the age of onset and compared. Twenty-two percent (N = 41) of the patients had pediatric-onset morphea (POLS) and 77.6% (n=142) had adult-onset morphea (AOLS). While POLS had a higher head-neck involvement, AOLS had a higher breast involvement (P < 0.001 and P = 0.043). Patients with linear morphea were younger, and more frequently had at least one laboratory anomaly (P = 0.016 versus 0.024). Anti-dsDNA positivity and low hemoglobin (Hb) were observed more frequently in patients with breast involvement. Patients with inguinal involvement, on the other hand, had lower Hb and a higher rate of diabetes, and those patients were older (P = 0.042, 0.040, and 0.012, respectively). Clinical characteristics and accompanying laboratory anomalies of the patients with morphea depend on the age of onset, involvement areas and the types of morphea, having such data readily available should guide the holistic approach for, and the monitoring process of, the disease.

Clinical and ultrasonographic evaluation of efficacy and safety of intralesional injection of autologous platelet-rich plasma in morphea: A comparative case series.

To evaluate the efficacy and safety of platelet-rich plasma to restore skin changes in morphea by ultrasound and Localized Scleroderma Cutaneous Assessment Tool. Nine morphea patients (21 lesions) were diagnosed clinically and by histopathology. Intradermal platelet-rich plasma was injected into morphea lesion once weekly for 12 sessions. The disease severity and damage were evaluated at baseline, after the last session (3 months later), and at 6 months follow-up using the LoSCAT and a high-resolution ultrasound. The healthy corresponding side was considered as a control. The Localized Scleroderma Cutaneous Assessment Tool score showed a significant improvement starting from 13 ± 7.28 up to 7.33 ± 6.82 after the therapeutic endpoint, reaching to 6.44 ± 7.09 after 6 months of follow-up with p value = 0.008 and 0.014, respectively. There was a significant positive correlation between the duration of the lesion and the improvement assessed by the ultrasound, with p value = 0.01. Regarding adverse effects, all patients reported having pain during platelet-rich plasma injection; transient edema of the face was reported by four patients (45%), and only two patients showed transient erythema. Autologous platelet-rich plasma is a safe technique with great aesthetic outcomes for filling up the contour defects and correcting both hyper and hypopigmentation, in addition to softening the indurated lesions.

Evaluation of Methotrexate Intolerance in Children With Morphea.

Methotrexate is an immunosuppressant commonly used in dermatology. The prevalence of intolerance using the Methotrexate Intolerance Severity Score (MISS) in pediatric juvenile idiopathic arthritis (JIA) ranges from 25% to 75%, but studies in morphea patients are lacking. We sought to determine the prevalence and predictors of methotrexate intolerance in children with morphea compared with children with inflammatory skin diseases and JIA/uveitis. Eligible patients were ages 2 to 18 years and were taking methotrexate for at least 3 months to treat morphea, inflammatory skin disease, or uveitis/JIA. Methotrexate intolerance was calculated using the MISS. A 1-way analysis of variance compared absolute intolerance scores. Multivariate regression analysis was used to compare MISS across diseases and covariates. Of 48 participants (mean ± SD age, 11.3 ± 4.1 years, 70.8% female), 15 had morphea, 16 had JIA/uveitis, and 17 had inflammatory skin diseases. The overall prevalence of intolerance was 20.8%. Age, sex, duration, and dose did not correlate with overall MISS. The MISS mean ± SD total for oral dosing was 2.5 ± 3.4, compared with 6.78 ± 6.8 for subcutaneous dosing. Patients with JIA/uveitis had the highest prevalence of intolerance (37.5%, n = 6), followed by morphea patients (20%, n = 3) and inflammatory skin disease patients (5.9%, n = 1). The OR of intolerance according to route of administration was 11.2 (95% CI, 2.03-61.89). Methotrexate intolerance was highest among patients with JIA/uveitis. The only predictor for risk of intolerance was subcutaneous route of administration. Future work could examine disease activity correlations and interventions designed to minimize intolerance.

Morphea, Eosinophilic Fasciitis and Cancer: A Scoping Review.

Morphea is an autoimmune fibrotic skin disease. Eosinophilic fasciitis (EF) is considered to belong to the severe spectrum of morphea. We conducted a scoping review assessing the risk of secondary cancer among morphea/EF patients, paraneoplastic morphea/EF and morphea/EF developing secondary to cancer therapy. The search was conducted using MEDLINE, Embase, Cochrane databases for articles published from inception to September 2022 following the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines with no language or date restrictions. Two hundred and one studies were included. Of these, 32 studies reported on secondary cancer in morphea/EF patients, 45 on paraneoplastic morphea/EF and 125 on cancer-treatment-induced morphea/EF. While the current evidence remains limited, data suggest an increased risk of secondary cutaneous and possibly pancreatic malignancy in morphea patients, particularly the generalized subtype. There were insufficient data for EF. On the other hand, paraneoplastic morphea was anecdotal, whereas several observational studies suggested that ~10% of EF cases may be paraneoplastic, primarily in the context of hematologic malignancies. Radiotherapy-induced morphea is rare, seen in ~0.2% of treated patients and is usually localized to the treatment site, except in patients with pre-existing autoimmunity. While chemotherapy-induced cases are reported, immunotherapy morphea/EF cases are emerging and are preferentially seen with PD-1 and not CTLA-4 inhibitors. This study is limited by the type of articles included (case reports, case series and observational studies), and hence, additional research on this important topic is needed.

Serum vitamin D levels and vitamin D receptor gene ApaI and TaqI polymorphisms in patients with morphea: a case-control study.

A uncommon inflammatory condition called morphea causes fibrosis in the skin and subcutaneous tissue. The key stages in the pathophysiology are vascular damage, immunological response, and fibrosis. Numerous research have examined the relationships between the immune system, fibrosis, and vitamin D, but the exact pathogenetic pathways of morphea remain poorly understood. The purpose of this study was to investigate serum 25(OH)D levels and the ApaI (rs7975232) and TaqI (rs731236) polymorphisms of the vitamin D receptor (VDR) in morphea patients. There were 48 age- and sex-matched controls and 41 morphea patients total. VDR polymorphisms were found using PCR tests and gel electrophoresis, and serum 25(OH)D levels were determined using liquid chromatography combined with tandem mass spectrometry (LC-MS/MS). The patient group consisted of 37 females (90.2%) and 4 males (9.8%). The patients' mean age was 38.68 ± 17.54years. In terms of VDR ApaI and TaqI polymorphisms, there was no discernible difference between the patient and control groups. TaqI polymorphism heterozygosity was discovered in all patients with progressive disease, and this finding was statistically significant (p = 0.012). Patients' mean serum 25(OH)D levels were 16.98 ± 11.55ng/mL, while those in the control group were 18.02 ± 14.30ng/mL. VDR polymorphisms, vitamin D levels, disease subtype, age of onset, and responsiveness to treatment did not significantly correlate. In our research, we discovered that TaqI polymorphism may be related to the severity of the disease and that the polymorphisms of the VDR ApaI and TaqI were not associated with morphea susceptibility.

Autoantigen microarrays reveal myelin basic protein autoantibodies in morphea

BackgroundMorphea is an autoimmune, sclerosing skin disorder. Despite the recent emphasis on immune dysregulation in morphea, the role of autoantibodies in morphea pathogenesis or utility as biomarkers are poorly defined.MethodsAutoantigen microarray was used to profile autoantibodies from the serum of participants from the Morphea in Adults and Children (MAC) cohort. Clinical and demographic features of morphea patients with myelin basic protein (MBP) autoantibodies were compared to those without. MBP immunohistochemistry staining was subsequently performed in morphea skin to assess for perineural inflammation in areas of staining. Immunofluorescence staining on mouse brain tissue was also performed using patient sera and mouse anti-myelin basic protein antibody to confirm the presence of MBP antibodies in patient sera.ResultsMyelin basic protein autoantibodies were found in greater frequency in morphea (n = 50, 71.4%) compared to systemic sclerosis (n = 2, 6.7%) and healthy controls (n = 7, 20%). Patients with MBP antibodies reported pain at higher frequencies. Morphea skin biopsies, highlighted by immunohistochemistry, demonstrated increased perineural inflammation in areas of MBP expression. Immunofluorescence staining revealed an increased fluorescence signal in myelinated areas of mouse brain tissue (i.e. axons) when incubated with sera from MBP antibody-positive morphea patients compared to sera from MBP antibody-negative morphea patients. Epitope mapping revealed target epitopes for MBP autoantibodies in morphea are distinct from those reported in MS, and included fragments 11–30, 41–60, 51–70, and 91–110.ConclusionsA molecular classification of morphea based on distinct autoantibody biosignatures may be used to differentially classify morphea. We have identified anti-MBP as a potential antibody associated with morphea due to its increased expression in morphea compared to healthy controls and systemic sclerosis patients.

26765 Myelin basic protein autoantibodies as a biomarker in morphea

The immune underpinnings of morphea are poorly described. Although numerous reports document autoantibodies associated with morphea, few have been replicated in large, controlled studies or are of clinical or mechanistic importance. We sought to characterize immunologic alterations and identify autoantibodies associated with morphea. We found increased frequency of CD3-CD138+ plasma cells and a decrease in CD19+CD138+ plasmablasts in morphea patients compared to controls, suggesting an alteration in the maturation, activity level, and function of antibody-secreting B cells of morphea patients.

28336 Outcomes of cosmetic procedures for treatment of morphea: A retrospective cohort study

Reconstructive surgery is recommended for cosmetic sequelae of morphea. We examined a retrospective cohort of 15 morphea patients who underwent a total of 41 procedures followed for 1-60 months postprocedure. The majority of patients were white (60%), female (60%), with facial linear morphea (93%). The most common procedure was fat grafting for facial morphea (75%, n = 30). Other procedures included gingival grafting (10%) and filler injections (7.5%). Recurrent disease activity was noted 8-36 months postprocedure in 4 patients, 3 after fat transfer and 1 after osteoplasty.

Serological Evidence of Borrelia burgdorferi in Patients with Morphea from West-Central Poland: An Original Paper and Review of Literature.

Morphea (localized scleroderma) is an inflammatory connective tissue disease. Borrelia burgdorferi, as a causative factor, has been discussed controversially. The aim of this original study was to evaluate the frequency of IgM and IgG classes of anti-Borrelia antibodies in groups of morphea and psoriasis patients using the traditional ELISA method. Blood samples of 82 patients with morphea and 112 patients with psoriasis vulgaris were examined for the presence of IgM and IgG classes of anti-Borrelia antibodies (ELISA). IgM and IgG classes of anti-Borrelia antibodies were detected in 4% of blood samples taken from morphea patients, while 4.5% of blood samples from patients with psoriasis vulgaris. There is one major limitation in this study that could be addressed in future research. First, the study focused on the determination of IgM and IgG classes of anti-Borrelia antibodies as a risk factor for morphea, but other infectious agents also require further testing, such as Hepatitis B, Hepatitis C, and other viral or bacterial infections. The results of this study showed no significant relationship between Borrelia infection and morphea. Detection of IgM and IgG classes of anti-Borrelia antibodies is not recommended for routine diagnostics of patients with morphea at this time.

571 Morphea in patients of color: A cross sectional study of the morphea in adults and children (MAC) cohort

Morphea is an autoimmune sclerosing skin condition. While clinical manifestations of morphea are well described in Caucasians, characteristics of morphea in patients of color have been poorly studied. Thus, the aim of this study was to determine the demographic and clinical characteristics of morphea in patients of color. This was an analysis of patients in the Morphea in Adults and Children Registry from 2007 to 2020. Kruskal-Wallis, Chi-square and Fischer’s exact tests were performed to investigate differences between racial groups. Post-hoc analysis was performed with Dunn’s test to directly compare individual racial groups. Of 772 patients in the registry, the majority had linear morphea (47%), and were white (73%). Overall, patients of color comprised 27% of the entire cohort, with 5% Black, 4% Asian, 14% Hispanic, and 4% Other. Age of morphea onset showed significant differences between racial groups (p=0.004). Black patients were more likely to have a later disease onset compared to non-Black patients and were less likely to have linear morphea (p=0.005). Asian and Hispanic patients were found to have similar subtype distribution to white patients. Years to diagnosis from disease onset also varied between racial groups (p=0.0058). Black and Hispanic morphea patients had a delayed diagnosis compared to White morphea patients [2.85 and 2.73 years in Black (p=0.015) and Hispanic (p=0.011) patients respectively vs 2.08 years in white patients]. Hispanic patients had higher disease damage by PGA-D, when compared to compared to White patients (Median: 30, IQR: 20-50 v. Median: 20, IQR: 10-40, p=<0.001). Our study demonstrates that morphea is infrequent in patients with skin of color, but still occurs in a substantial number of patients. We underscore the need for dermatologists to carefully consider distinct disease characteristics in patients of color, especially given the delay in diagnosis time experienced by Black and Hispanic patients compared to White patients.

698 Validation of CXCL9 as a biomarker in morphea

Morphea, also known as localized scleroderma, is a skin condition that has an initial presentation of inflammation followed by sclerosis. Clinical disease state (active versus inactive) is difficult to ascertain in morphea using clinical examination alone. To date, no biomarkers indicative of disease activity and/or severity have been validated in morphea. Biomarkers are needed to help guide clinical assessment of patients to determine 1) disease state, 2) subclinically active disease, and 3) early detection of recurrence of activity. Based on our preliminary studies, we hypothesize that interferon-gamma regulated chemokines, particularly CXCL9, may serve as useful biomarkers of morphea disease state. We analyzed baseline sera samples from 387 patients with morphea using enzyme-linked immunoassays to quantify CXCL9 levels. Analysis revealed morphea patients had elevated CXCL9 levels (median 81.6pg/mL, IQR 45.8-177.0) when compared to 26 age, race, and gender-matched controls (59.7pg/mL, IQR 44.1-79.8) (p=0.05). Importantly, CXCL9 levels were more highly elevated in those with active morphea (defined by a mLoSSI score >3) compared to inactive morphea, where median values were similar to that of controls (median 118.1 vs 59.7, IQR 53.8-278.3 vs 44.1-79.8) (p=0.003). CXCL9 also demonstrated correlation with clinical outcome measures indicative of an active disease state (activity component of the validated LoSCAT) (r=0.36, p<0.0001). Longitudinal analysis of 58 patients followed over a mean of 5 years with a median of 3 follow-up visits corroborated these results and also identified a correlation between elevated CXCL9 levels with increased age and greater functional involvement. Taken together, this study further validates CXCL9 as a biomarker for activity in morphea. Furthermore, it may be useful in monitoring for disease recurrence as elevations may occur prior to clinically evident activity. Consideration should be made to incorporate this measure as a supplement to clinical assessment.

AB012. Clinical and demographic features of morphea patients with mucocutaneous involvement: a cross sectional study from the morphea of adults and children (MAC cohort)

While many clinical findings of morphea have been described by our group and others, mucocutaneous findings, including genital or oral lesions, have not been well characterized. To date, we don’t know the frequency, demographic and clinical features associated with mucocutaneus lesions. To address this knowledge gap, we performed a cross-sectional analysis of patients enrolled in the Morphea in Adults and Children Registry from 2007 to 2018. Of the 737 patients analyzed, 48% (n=353) had linear morphea, 31% (n=232) had generalized morphea, 12% (n=87) had plaque morphea and 6% (n=45) had mixed. Oral lesions were present in 23 patients (3%), out of which 20 (87%) had linear morphea, nine had En Coup de Sabre (39%) and 12 had Parry Romberg syndrome (PRS) (52%). Genital lesions were present in 28 patients, the majority of which (24/28, 86%) had generalized morphea. Patients with oral involvement had a younger age of onset when compared to patients with genital involvement (12 and 58 years old, respectively; P<0.001). Seventy-nine percent (n=22) of morphea patients with genital involvement had lichen sclerosus et atrophicus (LsA) overlap, compared to 17% (n=4) with oral involvement (P<0.001). Eighty-three percent (n=19) patients with oral involvement were classified as having morphea profunda, or deep involvement, compared to 14% (n=4) of patients with genital involvement (P<0.001). Median mLoSSI ad PGA-A scores for patients with oral involvement (0, IQR 0–4 and 0, IQR 0–24, respectively)) was lower than patients with genital involvement (10, IQR 6–27 and 23, IQR 15–40, respectively) (P<0.001 and P=0.002, respectively). PGA-D scores were higher in patients with oral involvement (50, IQR 30–60) than in patients with genital involvement (20, IQR 10–25) (P<0.001). In conclusion, have demonstrated that mucocutaneous involvement of morphea lesions occurs most frequently in patients with linear and generalized morphea. Providers may not be aware of the prevalence of these findings and thus they may go undiagnosed for many months or years, leading to the development of potentially permanent and devastating sequalae.

Concentrations of metalloproteinase-1 in patients with morphea treated with phototherapy: a preliminary study.

Morphea (localized scleroderma) is a rare, chronic, inflammatory connective tissue disease, characterized by immune system dysfunction, vasculopathy and skin fibrosis. One of the most effective treatments is phototherapy. Phototherapy has been found to be effective in treating localized scleroderma by inducing the expression of metalloproteinase-1. To compare the concentrations of metalloproteinase (MMP-1) before psoralen and ultraviolet A (PUVA) and ultraviolet A1 (UVA1) treatments in the serum of patients with morphea. The observational study was conducted in one research centre and included patients with generalised morphea who were treated with PUVA and UVA1 phototherapies. The mean age of all morphea patients included in the study was 55.7 years. The levels of MMP-1 were examined by ELISA (The Biorbyt Human MMP-1 ELISA - Enzyme-Linked Immunosorbent Assay). The study showed that patients treated with PUVA and UVA1 had an improvement based on clinical measures, resulting in a reduction of clinical score. However, we did not observe statistically significant differences in MMP-1 concentrations before and after treatment. Limitations: The study sample was relatively small. Further studies on a larger group of patients would be beneficial. Our data suggest that there is a possible correlation between MMP-1 concentrations and phototherapy. MMP-1 levels were found to be increased following phototherapy treatment, which may suggest a correlation with better response to treatment in patients with morphea. However, further research is needed.

Effectiveness of PUVA vs. UVA1 phototherapy in the treatment of morphea patients.

IntroductionMorphea (localized scleroderma) is an inflammatory connective tissue disease, characterized by immune system dysfunction, vasculopathy and skin fibrosing. Phototherapy has been found to be effective in treating localized scleroderma. Psoralen + ultraviolet A (PUVA) and ultraviolet A1 (UVA1) phototherapy significantly enriched therapeutic possibilities.AimTo compare the clinical effect of PUVA photochemotherapy and UVA1 phototherapy and to evaluate the treatment response rates.Material and methodsIt was a retrospective one-centre research and observational study of all morphea patients treated with PUVA and UVA phototherapy. We reviewed phototherapy notes along with electronic and paper case records for all patients with morphea treated with PUVA and UVA1 phototherapy from January 2010 to December 2019.ResultsThe study shows that patients in both groups experienced improvement based on clinical measures, resulting in a reduction in the clinical score in all groups. There is positive short- and long-term efficacy of UVA1 and PUVA phototherapy in patients with morphea. There were no statistical differences between the treatment response rates. Limitations: We had a relatively small study sample and it was a retrospective, observational study.ConclusionsOur data suggest that ultraviolet PUVA and UVA1 should be considered for the treatment of morphea with disseminated lesions or not responding to topical treatment. UVA1 is free of side effects linked to oral psoralens such as nausea, vomiting, photokeratosis, but we showed that there was no statistical advantage in the effectiveness of both. UVA1 phototherapy is, however, a less accessible form of treatment, available in the centres of higher quality.

18148 Myelin basic protein autoantibodies as a biomarker in morphea

The immune underpinnings of morphea are poorly described. Although numerous reports document autoantibodies associated with morphea, few have been replicated in large, controlled studies or are of clinical or mechanistic importance. We sought to characterize immunologic alterations and identify autoantibodies associated with morphea. We found increased frequency of CD3−CD138+ plasma cells and a decrease in CD19+CD138+ plasmablasts in morphea patients compared with controls, suggesting an alteration in the maturation, activity level, and function of antibody-secreting B cells of morphea patients.

Morphea patients with mucocutaneous involvement: A cross-sectional study from the Morphea in Adults and Children (MAC) cohort

Demographic and clinical findings of patients with mucocutaneous morphea have not been well characterized, to our knowledge. To determine the demographic and clinical characteristics of morphea patients with mucocutaneous lesions who were enrolled in the Morphea in Adults and Children cohort. Cross-sectional study of 735 patients in the Morphea in Adults and Children cohort from 2007 to 2018. A total of 4.6% of linear morphea patients had oral involvement versus 2.4% among the entire cohort, whereas 10.3% of generalized morphea patients had genital involvement versus 3.7% among the entire cohort. Patients with genital lesions were older at disease onset than those with oral morphea (57 versus 11.5years; P<.001) and had more frequent extragenital lichen sclerosus et atrophicus (59.2% versus 5.6%; P=.004). Selection bias and limited number of affected subjects. Oral morphea lesions predominate in younger patients with facial linear morphea, whereas genital lesions predominate in postmenopausal women with overlying extragenital lichen sclerosus et atrophicus.

Comparison of the tissue expressions of glutathione S transferase isoenzymes among patients with morphea and healthy controls: A preliminary study.

Morphea is an inflammatory connective tissue disorder, which is characterized by sclerosis in skin and subcutaneous tissues with a chronic progress. The oxidative stress in pathogenesis of sclerosing diseases was proposed in several studies with conflicting results. To explore the tissue expressions of Glutathione S transferase (GST) isoenzymes in patients with morphea and compare these expressions with healthy controls. Twenty-two morphea patients and 20 sex and age matched healthy controls were enrolled in this study. Four millimeter punch biopsies were performed from the active sclerotic plaques of morphea patients. Tissue samples of control group were obtained from nonlesional normal skin biopsy specimens. The protein expressions of GST isoenzymes were analyzed immunohistochemically. Tissue expressions of GSTP1, GSTT1, and GSTA1 isoenzymes in morphea patients were found to be significantly higher than in control tissues. There was no significant difference in GSTM1 isoenzyme expression between the two groups. The increased tissue expressions of GSTA1, GSTP1, and GSTT1 isoenzymes in morphea may represent the activated GST enzymes in response to excessive free radical formation and may also support the hypothesis of increased oxidative stress in morphea etiopathogenesis.

Evaluation of anxiety and depression in patients with morphea taking immunosuppressive drugs during the COVID-19 pandemic

Background During the coronavirus disease 2019 (COVID-19) pandemic, individuals especially those with chronic diseases face various problems like psychological distress. Aim To evaluate anxiety and depression among morphea patients taking immunosuppressants compared with controls during the COVID-19 pandemic. Methods In this case-control study, electronic data of 42 eligible morphea patients and 42 age- and sex-matched healthy subjects were extracted. All participants were asked about adherence to the health protocols and the symptoms of COVID-19. To investigate anxiety and depression, we provided the participants with an online version of the Hospital Anxiety and Depression Scale (HADS) Questionnaire. In this standard questionnaire, a score ≥ 11 represents a significant psychological disorder. Results The two groups did not differ significantly in the mean HADS-Anxiety (HADS-A) and HADS-Depression (HADS-D) scores (p= .08 and p= .79, respectively). HADS-A and HADS-D scores were ≥ 11 in 16.67% and 11.90% of the patients, respectively. Among the morphea patients, those with sleeping problems during COVID-19 had a significantly higher mean HADS-D score (p= .048). Notably, 21.43% of patients discontinued their treatment due to fear of COVID-19 and experienced disease recurrence. Adherence to health protocols was higher in patients compared with controls. The rate of COVID-19 occurrence was not significantly different between the groups. Conclusions Although there was no significant difference in anxiety and depression between the groups, it is recommended to provide psychiatric counseling opportunities to morphea patients to improve treatment outcomes during this pandemic.