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 if untreated. Research exploring 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 severity of morphea. It focused on assessing the levels of RF among morphea patients and its correlation with disease severity, intending to provide deeper insights into the 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 Hospital from 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 showing higher 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.

High-frequency ultrasound evaluation of morphea: Retrospective analytical study.

BACKGROUND: To date, there are no accepted outcome measures to monitor morphea, and consensus on specific monitoring criteria for morphea remains elusive. A few studies have assessed the criterion validity of skin ultrasound in morphea. So, in this study, we approach ultrasound findings in morphea lesions. MATERIAL AND METHODS: This was a retrospective-analytical study conducted between December 2021 and May 2023. Patients were clinically evaluated at a dermatology outpatient clinic and then referred for high-frequency ultrasound (HF-US) evaluation and were selected to be included in this study. The lesions were confirmed by histopathology as well. Sonographic evaluations were performed on the lesion site and the symmetrical uninvolved other side. Dermal thickness and dermal echogenicities were recorded. Statistical analysis of group differences was performed by using the 2-tailed Student t-test. A p-value of less than 0.05 was considered statistically significant. RESULTS: Forty-one morphea lesions in the inflammatory phase of 27 patients were included in the study. The mean dermal thickness of morphea lesions was 1107.97 ± 414.3 and the mean dermal thickness of the control side was 1094.65 ± 331.06, The difference between these two variables was not statistically significant. The mean dermal density of lesions was 49.13 ± 18.97 and the mean dermal density of the control side was 52.22 ± 25.33. The difference between these two variables was not statistically significant. CONCLUSION: This study shows that HF-US indicated increasing dermal thickness and reducing the dermal density of the morphea lesions in the inflammatory phase confirmed with the histopathology.

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

Objective: To evaluate the efficacy and safety of platelet-rich plasma to restore skin changes in morphea by ultrasound and Localized Scleroderma Cutaneous Assessment Tool. Methods: 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. Results: 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. Conclusion: 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.

Scleroderma and scleroderma-like syndromes.

Systemic sclerosis is a systemic connective tissue disease whose main pathophysiological mechanism is a progressive fibrosis of internal organs and skin leading to thickening and induration. Blood vessels may also be involved. However, systemic scleroderma is not the only disease causing cutaneous sclerosis. There is a group of diseases that mimic scleroderma in their clinical presentation - these are scleroderma-like syndromes. A distinction can be made between syndromes of inflammatory/autoimmune, genetic, metabolic, toxic, drug-induced, occupational, paraneoplastic and syndromes caused by deposition disorders. In the following paper, we have reviewed the literature on scleroderma-like syndromes. We have outlined the factors predisposing to the development of each disease, its pathogenesis, clinical presentation, diagnostic and treatment process and the differences between each syndrome and systemic scleroderma.

Skin Hypopigmentation in Hematology Disorders.

Hypopigmentation disorders pose significant diagnostic challenges in dermatology, sometimes reflecting underlying hematological conditions. This review explores the clinical presentations related to hypopigmentation in hematological disorders, focusing on vitiligo, morphea, and syndromic albinism. Vitiligo, an autoimmune disorder targeting melanocytes, involves interactions between genetic polymorphisms and immune responses, particularly regarding CD8+ T cells and IFN-γ. Drug-induced vitiligo, notably by immune checkpoint inhibitors and small-molecule targeted anticancer therapies, underscores the importance of immune dysregulation. Morphea, an inflammatory skin disorder, may signal hematological involvement, as seen in deep morphea and post-radiotherapy lesions. Syndromic albinism, linked to various genetic mutations affecting melanin production, often presents with hematologic abnormalities. Treatment approaches focus on targeting the immune pathways specific to the condition, and when that is not possible, managing symptoms. Understanding these dermatological manifestations is crucial for the timely diagnosis and management of hematological disorders.

Nodular/Keloidal Scleroderma with No Systemic Involvement-A Case Report and a Review of the Literature.

Nodular or keloidal scleroderma is a rare condition with unclear cause and sporadic mentions in the medical literature. It was first recognized in the 19th century, yet its classification is still debated due to the limited number of reported cases. This rare variant of scleroderma is associated with either progressive systemic sclerosis or localized morphea. Clinically, it presents with asymptomatic nodules or plaques, resembling spontaneous keloid formation, often found on the trunk and proximal extremities. Recent literature reviews show a predominance of women with a mean age of 44 years. Diagnosis relies on clinical and histopathological findings, which usually show overlapping features of both scleroderma and true keloids, secondarily to an excessive fibrosing reaction attributed to collagen formation. We present an unusual case of a 70-year-old female patient who displayed the coexistence of two distinct subtypes of morphea (nodular/keloidal and linear), and exclusive skin involvement, which contrasts with the typical presentation of nodular/keloidal scleroderma, often associated with organ-specific disease. However, recent publications have diverged from previous ones regarding systemic sclerosis, with no systemic involvement reported between 2018 and 2024, which we evaluated in our descriptive literature review. With less than 50 cases reported in total, our case underlines the importance of recognizing this rare disease, ensuring appropriate evaluation, treatment, and follow-up.

Linear Morphea in the Context of Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) Infection: A Unique Dermatological Manifestation.

Morphea, a rare skin disorder characterized by localized areas of thickened and sclerotic skin, typically presents as circumscribed plaques. The linear variant, however, manifests as linear bands of sclerosis affecting the extremities, and its association with coronavirus disease 2019 (COVID-19) has not been documented until now. In this article, we present the case of a 22-year-old previously healthy female patient who contracted COVID-19 complicated by an erythroedema on the back of the right hand, extending notably to the forearm on the 10th day of the infection. Skin biopsy revealed dermal and septal hypodermal fibrosis with a mild lymphocytic interstitial infiltrate in the dermis consistent with morphea. Treatment with low-dose corticosteroids was started, and regular follow-up was established. An isolated recurrence of cutaneous symptoms was observed after the first COVID-19 vaccination (Sputnik V) administered five months after the initial infection, with spontaneous regression in 10 days. This clinical evolution underscores the importance of a comprehensive understanding of dermatological manifestations in COVID-19, particularly in the context of post-infection vaccination.

Linear indurated plaque over the neck.

Isolated cutaneous swelling can have varied etiologies. The clinical diagnosis is usually difficult, and a correct diagnosis always requires a pathological examination. Hereby, we report a case of linear keloidal morphea on the neck of an 18-year-old male who presented with an asymptomatic, firm lesion for 6 months. Histopathological examination was consistent with morphea. This case highlights the uncommon form of morphea in an unusual location, which can be misdiagnosed for numerous neoplastic conditions and for which simple histopathological evaluation can clinch the diagnosis.

Hemimasticatory spasm: a series of 17 cases and a comprehensive review of the literature.

Hemimasticatory spasm (HMS) is a rare movement disorder characterized by paroxysmal spasms or twitches of the unilateral jaw-closing muscles. This study aimed to comprehensively evaluate the clinical features of patients with HMS. Data from 17 patients newly diagnosed with HMS (12 females and 5 males; mean age at onset: 46.7 years) who visited our department were retrospectively analyzed, and a literature search based on electronic medical databases from their inception until November 30, 2023, was conducted. A manual search was conducted for articles cited in the related literature. A total of 117 cases (72 females and 45 males; mean age at onset: 37.1 years) from 57 studies were analyzed. The muscles involved were the masseter (97.4%), temporalis (47.9%), and medial pterygoid (6%). Morphea or scleroderma was observed in 23.9% of the patients, and facial hemiatrophy in 27.4%. In 17.9% of the cases, Parry-Romberg syndrome was either complicated or suspected. Typical electromyographic findings included the absence of a silent period during spasms (23.9%) and irregular brief bursts of multiple motor unit potentials. Oral medicines, such as clonazepam or carbamazepine, alleviated the symptoms for some patients but were often unsatisfactory. Botulinum toxin therapy was effective in most cases. Recently, microvascular decompression surgery is increasingly being used, resulting in complete relief in some cases. In conclusion, highly effective modalities are currently available, and it is necessary to raise awareness of HMS to ensure that it can be diagnosed and treated accurately by both medical and dental professionals.

A difficult diagnosis of eosinophilic fasciitis: A case report.

Eosinophilic fasciitis can be a debilitating diagnosis and is often delayed given its similarities to other sclerotic conditions including morphea, such as bound-down indurated skin and inflammation and sclerotic thickening of tissue layers on histopathology. Delaying treatment can lead to joint contracture and residual hardness in skin which has both cosmetic and functional implications. Therefore, finding the definitive diagnosis and differentiating from other sclerotic diseases is important early in the disease course. We present a case of a 77-year-old female with a generalized rash on her back and extremities, and progressive symptoms of pain, joint contractures, and limited movement, which highlights the challenges in diagnosis and management given clinical and histological parallels between eosinophilic fasciitis and morphea.

S2k guideline: Diagnosis and therapy of localized scleroderma.

The updated S2k guideline deals with the diagnosis and therapy of localized scleroderma (LoS). LoS represents a spectrum of sclerotic skin diseases in which, depending on the subtype and localisation, structures such as adipose tissue, muscles, joints, and bones may also be affected. Involvement of internal organs or progression to systemic sclerosis does not occur. LoS can be classified into four main forms: limited, generalized, linear, and mixed forms, with some additional subtypes. For cases of limited skin involvement, the guideline primarily recommends therapy with topical corticosteroids. UV therapy can also be recommended. In subtypes with severe skin or musculoskeletal involvement, systemic therapy with methotrexate is recommended. During the active phase of the disease, systemic glucocorticosteroids can be used additionally. In cases of methotrexate and steroid refractory courses, contraindications, or intolerance, mycophenolate mofetil, mycophenolic acid, or abatacept can be considered as second-line systemic therapies. In the case of linear LoS, autologous adipose-derived stem cell transplantation can also be performed for correcting soft tissue defects.

Low-Powered View, High-Powered Mind: A Case Report of Morpheaform Basal Cell Carcinoma Mistaken for Metastatic Breast Carcinoma.

Basal cell carcinoma (BCC) is one of the most common skin malignancies worldwide. Morpheaform basal cell carcinoma (MBCC) is a rare aggressive subtype of BCC that presents with unique histologic features. Both are treated surgically and have an excellent survival rate. Metastatic breast carcinoma, on the other hand, has a poor survival rate along with a more burdensome therapeutic route including chemotherapy. Due to an overlap in common immunohistochemistry stains, there is a possibility of confusing the diagnosis of BCC with metastatic breast carcinoma resulting in potential patient harm. Therefore, a timely and accurate diagnosis distinguishing these malignancies is essential. We report a near-miss event in which a 77-year-old female with MBCC was mistakenly diagnosed with metastatic breast carcinoma. We discuss the details of these stains, characteristic features of MBCC, and treatment options and emphasize the importance of combining laboratory medicine with clinical expertise to improve patient outcomes.

Damage evaluation of craniofacial localized scleroderma using magnetic resonance imaging.

Background: Localized scleroderma (LoS) is an autoimmune disease in which craniofacial lesions can cause severe facial deformities with brain involvement. Objective evaluation of craniofacial LoS is challenging. Magnetic resonance imaging (MRI) may be used as a damage assessment tool. This study aimed to analyze the tissue involvement of craniofacial LoS based on MRI and evaluate MRI for craniofacial LoS assessment. Methods: This cross-sectional study included patients with craniofacial LoS from September 2021 to August 2022 in Peking Union Medical College Hospital. Patients who were clinically assessed in a stable phase were enrolled; patients with previous surgical treatment or contraindications to MRI were excluded. Participants underwent clinical, MRI, and ultrasound assessments. MRI was compared with ultrasound by correlation analysis and Bland-Altman analysis. The involvement of different tissues and different facial subunits was compared. The accumulated soft tissue atrophy index (ASTAI) was compared with clinical scores by correlation analysis. Results: A total of 28 patients were included (13 female; mean age, 18 years). MRI showed a good correlation and agreement with ultrasound (r=0.916, P<0.001). In different facial subunits, a significant negative correlation between the forehead and chin was found (r=-0.593, P=0.001). The ASTAI correlated well with the facial LoS damage index (r=0.580, P=0.001) and the Peking Union Medical College LoS facial aesthetic index (PUMC LoSFAI) (r=0.921, P<0.001). A total of 38.6% of clinical scores were inaccurate based on MRI. Neurological changes were found in one patient. Conclusions: MRI can reliably quantify damage in craniofacial LoS, and may serve as a useful and objective tool for overall craniofacial LoS evaluation.