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1.
Dermatol Reports ; 16(3): 9915, 2024 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-39290556

RESUMO

Morphea is a rare autoimmune disease that often affects skin and subcutaneous tissues. The aim of this study was to determine the association between patient demographic parameters, lesion site, clinical subtype of morphea, and histological findings. Between 2016 and 2022, we investigated 78 patients with morphea at the Department of Pathology, Prof. Dr. Cemil Tascioglu City Hospital in Turkey. Case-specific hematoxylin and eosin stain slides were obtained from the pathology archive and assessed blindly by two pathologists. Flattening of rete ridges, location of inflammatory infiltrate, grade of inflammatory infiltrate, presence of plasma cells, presence of eosinophils, homogenization of dermal collagen, decrease of skin appendages, basal pigmentation and melanin incontinence were evaluated. Statistical analyses were performed using SPSS Statistics v.20 (IBM, Armonk, NY, USA). The most common clinical presentation was plaque type (87.5%), while histopathological findings included homogenization of dermal collagen (100%) and decrease of skin appendages (98.7%). Flattening of the rete ridges was observed in 46.2% of patients. Severity of the inflammatory infiltrate was found to be higher in these patients (p=0.028). Basal pigmentation was observed in 59% of patients. Line sign was more common in lower extremity lesions among all localizations (p=0.015). The histopathologic features of morphea are variable and confusing. Particularly, in cases with collagen homogenization, morphea should be considered in differential diagnosis with clinical correlation. In addition, the line sign could be helpful for identifying lesions located in the lower extremities.

2.
Arch Dermatol Res ; 316(8): 611, 2024 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-39259320

RESUMO

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.


Assuntos
Neoplasias , Esclerodermia Localizada , Humanos , Masculino , Feminino , Esclerodermia Localizada/epidemiologia , Esclerodermia Localizada/diagnóstico , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto , Idoso , Neoplasias/epidemiologia , Neoplasias/diagnóstico , Incidência , Comorbidade , Adulto Jovem
3.
Front Surg ; 11: 1457593, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39247703

RESUMO

Background: The application of autologous fat transplantation in facial lesions of patients with localized scleroderma (LoS) has been reported in recent years. Objective: The authors report a case of worsening of active localized scleroderma after autologous fat transplantation. Methods: A man presented with neck and facial skin atrophy and pigmentation with a history of LoS. Appearing 1.5 years ago, the lesion had progressively grown in size and shape. Consent was obtained after the patient was informed of the possible surgical risks during the active phase of the disease. He underwent autologous fat grafting into the right cheek with about 30 ml Coleman fat graft. Results: Skin dyspigmentation and atrophy progressively deteriorated 1 month into therapy, with slightly increased erythema and enlargement of the lesion. Six months after the therapy, the localized scleroderma-related score worsened. Limitations: There are different factors, such as that systemic medications could affect the treatment of localized scleroderma by autologous fat transplantation. Meanwhile, considering the limitation of the 6-month follow-up period, obtaining long-term follow-up data is necessary to evaluate sustained outcomes and potential complications. Conclusion: More clinical research is needed to determine the time interval between disease inactivity and the application of any surgical procedures to avoid reactivation.

4.
Cureus ; 16(7): e65423, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-39184775

RESUMO

Morphea, a form of localized scleroderma, can significantly affect individuals by causing skin tightening and discoloration. We describe the case of a 22-year-old woman who presented with progressive skin changes and discomfort in her right gluteal region following a history of an intramuscular injection in the right gluteal region. Clinical examination suggested morphea, prompting us to conduct an MRI to better understand the extent and nature of her condition. The MRI results revealed thickening of the skin layers and signs of inflammation, helping us differentiate between active inflammation and fibrosis. This case illustrates how MRI can provide crucial insights for managing morphea effectively.

5.
Pediatr Rheumatol Online J ; 22(1): 77, 2024 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-39169409

RESUMO

BACKGROUND: Current treatment for localized scleroderma (LS) has been shown to halt disease activity, but little is still known about patient experiences with these treatments, nor is there consensus about optimal measurement strategies for future clinical trials. OBJECTIVE: Conduct a scoping review of the literature for the types of outcomes and measures (i.e. clinician-, patient-, and caregiver-reported) utilized in published treatment studies of LS. METHODS: Online databases were searched for articles related to the evaluation of treatment efficacy in LS with a special focus on pediatrics. RESULTS: Of the 168 studies, the most common outcomes used were cutaneous disease activity and damage measured via clinician-reported assessments. The most frequently cited measure was the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT). Few patient-reported outcome measures (PROMs) were used. LIMITATIONS: Some studies only vaguely reported the measures utilized, and the review yielded a low number of clinical trials. CONCLUSION: In addition to evaluating disease activity with clinician-reported measures, the field could obtain critical knowledge on the patient experience by including high-quality PROMs of symptoms and functioning. More clinical trials using a variety of outcomes and measures are necessary to determine the most suitable course of treatment for LS patients.


Assuntos
Medidas de Resultados Relatados pelo Paciente , Esclerodermia Localizada , Humanos , Esclerodermia Localizada/terapia , Esclerodermia Localizada/diagnóstico , Resultado do Tratamento , Criança , Avaliação de Resultados em Cuidados de Saúde
6.
J Pers Med ; 14(8)2024 Aug 17.
Artigo em Inglês | MEDLINE | ID: mdl-39202063

RESUMO

Localized scleroderma (LS), commonly known as morphea, presents a significant clinical challenge due to its chronic, inflammatory nature affecting the skin and potentially underlying tissues. This systematic review explores the innovative approach of combining laser therapy and injectable fillers, specifically hyaluronic acid, for the treatment of LS. We conducted a comprehensive literature review following PRISMA guidelines, examining articles from MEDLINE/PubMed to assess the combined efficacy of these treatments in improving both esthetic and functional outcomes for LS patients. The search yielded 64 articles, with six selected for in-depth analysis for a total of nine patients, covering a range of patient demographics and treatment types. Our review highlights cases where fractional CO2 laser therapy promoted long-term tissue remodeling and instances where hyaluronic acid fillers effectively addressed skin atrophy and volume loss, enhancing both immediate and long-lasting esthetic improvements. The synergy between these treatments suggests a promising dual approach, aiming to maximize esthetic outcomes and to improve the quality of life for LS patients. This review underscores the necessity of further research to establish a comprehensive, evidence-based clinical pathway integrating both treatments for managing LS, thereby enhancing patient satisfaction and addressing the multifaceted nature of this challenging dermatological condition.

7.
Cell Mol Biol Lett ; 29(1): 115, 2024 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-39215271

RESUMO

BACKGROUND: Localized scleroderma (LoS) is an autoimmune disorder that primarily affects the skin, and is often treated with autologous fat grafting (AFG). Nevertheless, the retention rate of AFG in patients with LoS is typically low. We hypothesize that the low retention rate may be partially attributed to the inherent abnormalities of adipose-derived stem cells (ASCs) from nonlesional sites of patients with LoS. METHODS: We performed a comparative analysis of the single-cell transcriptome of the SVF from nonlesional sites of patients with LoS and healthy donors, including cellular compositional analysis, differential expression analysis, and high-dimensional weighted gene coexpression network analysis. Experimental validation with fluorescence-activated cell sorting and bleomycin-induced skin fibrosis mice models were conducted. RESULTS: We found a significant reduction in the relative proportion of CD55high interstitial progenitors in ASCs under the condition of LoS. Differential expression analysis revealed inherent abnormalities of ASCs from patients with LoS, including enhanced fibrogenesis, reduced anti-inflammatory properties, and increased oxidative stress. Compared with CD55low ASCs, CD55high ASCs expressed significantly higher levels of secreted protein genes that had functions related to anti-inflammation and tissue regeneration (such as CD55, MFAP5, and METRNL). Meanwhile, CD55high ASCs expressed significantly lower levels of secreted protein genes that promote inflammation, such as chemokine and complement protein genes. Furthermore, we provided in vivo experimental evidence that CD55high ASCs had superior treatment efficacy compared with CD55low ASCs in bleomycin-induced skin fibrosis mice models. CONCLUSIONS: We found that the low retention rate of AFG may be partially ascribed to the reduced pool of interstitial progenitor cells (CD55high) present within the ASC population in patients with LoS. We demonstrated the potential for improving the efficacy of AFG in the treatment of LoS by restoring the pool of interstitial progenitors within ASCs. Our study has significant implications for the field of translational regenerative medicine.


Assuntos
Tecido Adiposo , Esclerodermia Localizada , Análise de Célula Única , Células-Tronco , Humanos , Animais , Esclerodermia Localizada/genética , Esclerodermia Localizada/patologia , Camundongos , Células-Tronco/metabolismo , Células-Tronco/citologia , Tecido Adiposo/citologia , Tecido Adiposo/metabolismo , Feminino , Análise de Sequência de RNA/métodos , Transcriptoma/genética , Adulto , Modelos Animais de Doenças , Masculino , Camundongos Endogâmicos C57BL , Pele/patologia , Pele/metabolismo , Pessoa de Meia-Idade , Fibrose
8.
Pediatr Dermatol ; 2024 Jul 09.
Artigo em Inglês | MEDLINE | ID: mdl-38982207

RESUMO

Morphea, also known as localized scleroderma, is an inflammatory sclerosing disorder of uncertain pathogenesis that affects the skin and underlying tissues. In the pediatric population, the disease often runs a chronic course with a high risk for irreversible sequelae; as such, patients often require long-term monitoring. The objective of this study is to develop a multi-center, consensus-based electronic medical record template for pediatric morphea patient visits using a modified Delphi method of iterative surveys. By facilitating consistent data collection and interpretation across medical centers and patient populations, this template may improve patient care for pediatric patients with morphea.

9.
J Scleroderma Relat Disord ; 9(2): 162-168, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38910599

RESUMO

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.

10.
Skin Res Technol ; 30(7): e13818, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38924131

RESUMO

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.


Assuntos
Esclerodermia Localizada , Ultrassonografia , Humanos , Esclerodermia Localizada/diagnóstico por imagem , Esclerodermia Localizada/patologia , Estudos Retrospectivos , Feminino , Masculino , Ultrassonografia/métodos , Adulto , Pessoa de Meia-Idade , Adolescente , Adulto Jovem , Pele/diagnóstico por imagem , Pele/patologia , Criança
12.
Front Immunol ; 15: 1405215, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38868763

RESUMO

Chronic inflammatory skin diseases are multifactorial diseases that combine genetic predisposition, environmental triggers, and metabolic disturbances associated with abnormal immune responses. From an immunological perspective, the better understanding of their physiopathology has demonstrated a large complex network of immune cell subsets and related cytokines that interact with both epidermal and dermal cells. For example, in type-1-associated diseases such as alopecia areata, vitiligo, and localized scleroderma, recent evidence suggests the presence of a type-2 inflammation that is well known in atopic dermatitis. Whether this type-2 immune response has a protective or detrimental impact on the development and chronicity of these diseases remains to be fully elucidated, highlighting the need to better understand its involvement for the management of patients. This mini-review explores recent insights regarding the potential role of type-2-related immunity in alopecia areata, vitiligo, and localized scleroderma.


Assuntos
Vitiligo , Humanos , Vitiligo/imunologia , Animais , Alopecia em Áreas/imunologia , Células Th2/imunologia , Citocinas/metabolismo , Citocinas/imunologia , Dermatite Atópica/imunologia , Dermatite Atópica/etiologia , Esclerodermia Localizada/imunologia , Inflamação/imunologia , Pele/imunologia , Pele/patologia
13.
Curr Med Imaging ; 20: e15734056299155, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38798225

RESUMO

BACKGROUND: Although clinical assessment has historically been the primary method used for pediatric localized sclerosis (LS) diagnosis and staging, highfrequency ultrasonography (HFUS) is being investigated as a more accurate evaluation method for lesion. OBJECTIVES: This study aimed to assess, compare dermal and subcutaneous tissue characteristics and enhance enhance lesion staging in pediatric LS patients using HFUS. METHODS: Twenty two LS patients were cross-sectionally evaluated with B-mode ultrasonography. Lesions were clinically staged, and dermal and subcutaneous tissue characteristics were compared with healthy tissue using HFUS. RESULTS: Among 55 lesions, 27 were active/new (49.1%), and 28 were atrophic/old (50.9%). Active lesions typically had increased dermal thickness in 66.6% of cases, while atrophic lesions often showed decreased dermal thickness (78.5%), with significant differences (p<0.05). Dermal echogenicity decreased in 40.7% of active lesions but remained largely unchanged in atrophic lesions (82.1%) (p<0.05). Subcutaneous tissue thickness significantly decreased in atrophic lesions (78.5%) and increased in 59.2% of active lesions, with a significant difference (p = 0.002). Subcutaneous tissue echogenicity increased in 44.4% of active lesions and remained mostly unchanged in atrophic lesions (67.8%). Importantly, a considerable proportion of lesions diagnosed as active through physical examination were actually inactive on HFUS evaluation (55.6%), while a significant portion of lesions categorized as atrophic on physical examination displayed areas of inactivity upon ultrasonographic assessment (35.7%). These findings highlight HFUS's potential as a valuable diagnostic tool and reveal discordances between clinical and HFUS staging. CONCLUSION: Ultrasonography offers an objective LS lesion evaluation, especially in pediatrics.


Assuntos
Esclerodermia Localizada , Pele , Ultrassonografia , Esclerodermia Localizada/diagnóstico por imagem , Pele/diagnóstico por imagem , Pele/patologia , Ultrassonografia/métodos , Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Tela Subcutânea/diagnóstico por imagem , Voluntários Saudáveis , Estudos Transversais
14.
Cureus ; 16(3): e56990, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38681456

RESUMO

The autoimmune connective tissue disease scleroderma is characterized by fibrosis of the skin, blood vessels, and visceral organs. Overlap syndromes are conditions in which a patient has characteristics from two or more autoimmune disorders. The coexistence of scleroderma and lupus nephritis is rare but documented. However, it is crucial to note that both scleroderma and lupus are complex autoimmune diseases with diverse clinical presentations and can affect multiple organ systems, including the kidneys. This case report presents a unique clinical scenario of a patient with the coexistence of scleroderma and systemic lupus erythematosus with Class V lupus nephritis, highlighting the challenges in diagnosis, treatment, and the need for a multidisciplinary approach.

15.
J Dtsch Dermatol Ges ; 22(4): 605-620, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38426689

RESUMO

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.


Assuntos
Fármacos Dermatológicos , Esclerodermia Localizada , Humanos , Metotrexato/uso terapêutico , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/terapia , Pele , Fármacos Dermatológicos/uso terapêutico , Ácido Micofenólico/uso terapêutico
16.
Pediatr Rheumatol Online J ; 22(1): 29, 2024 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-38395889

RESUMO

BACKGROUND: Eosinophilic fasciitis (EF) is a rare disease characterized by skin induration and musculoskeletal abnormalities. Diagnostic criteria for EF are based on adult populations. There is a need to expand the literature on EF in children due to limited reported cases and potential differences compared to adults. METHODS: We conducted a retrospective review of medical records for six pediatric patients diagnosed with EF at our institution between November 2011 and April 2023. Inclusion criteria required patients to be under 18 years of age at the time of diagnosis and to have confirmed diagnosis through clinical history, imaging, and histology. RESULTS: Most of our cohort were female (83%) and non-Hispanic white (50%). Age at diagnosis ranged from 4 to 16 years. Duration of symptoms before diagnosis varied from 1 to 12 months. Follow-up periods ranged from 14 to 123 months. Concurrent medical conditions included localized scleroderma, acquired thrombophilia, and juvenile idiopathic arthritis. Patients presented with progressive painful swelling, severe joint limitations, and positive prayer sign. Initial regimens involved corticosteroids and methotrexate. Hydroxychloroquine, immunoglobulin, mycophenolate mofetil, rituximab, and tocilizumab were also used depending on the patient's disease severity and course. CONCLUSIONS: Juvenile EF may manifest as swelling and progressive induration without apparent skin abnormalities. Unlike adult populations, no underlying malignancies or associations with trauma were observed in our cohort. Our cases did not exhibit systemic involvement observed in previous studies on juvenile EF. While non-specific, the prayer sign may aid in early recognition of juvenile EF and help prevent long-term disability.


Assuntos
Eosinofilia , Fasciite , Adulto , Humanos , Criança , Feminino , Adolescente , Pré-Escolar , Masculino , Diagnóstico Diferencial , Fasciite/diagnóstico , Fasciite/tratamento farmacológico , Fasciite/complicações , Metotrexato/uso terapêutico , Eosinofilia/diagnóstico , Eosinofilia/tratamento farmacológico , Eosinofilia/complicações
17.
Quant Imaging Med Surg ; 14(2): 1891-1903, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38415131

RESUMO

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.

18.
Dermatologie (Heidelb) ; 75(3): 208-213, 2024 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-38240812

RESUMO

Juvenile scleroderma, often referred to as juvenile localized scleroderma or "morphea", is a rare inflammatory disease of the skin and skin-related structures, accompanied by local sclerosis and tissue fibrosis. Depending on the clinical manifestation, four different subtypes can be defined: limited, generalized, linear, and mixed. To prevent possible sequelae of the disease, the diagnosis should be made as early as possible and therapy should be initiated at specialized centers in multiprofessional pediatric and dermatologic collaboration. In this review, we present the main clinical, laboratory, and therapeutic characteristics of juvenile localized scleroderma and summarize recommendations.


Assuntos
Esclerodermia Localizada , Escleroderma Sistêmico , Humanos , Criança , Esclerodermia Localizada/diagnóstico , Escleroderma Sistêmico/diagnóstico , Pele/patologia , Fibrose , Progressão da Doença
19.
Curr Rheumatol Rev ; 20(4): 451-454, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38243962

RESUMO

BACKGROUND: Tumor necrosis factor alpha (TNFα) is a pivotal cytokine involved in the pathogenesis of certain inflammatory diseases, such as rheumatoid arthritis (RA), spondyloarthropathies, and inflammatory bowel diseases. In the last two decades, TNFα inhibitors (TNFi) have revolutionized the treatment and outcome of the above disorders. However, the use of TNFi has been associated with the development of many autoimmune phenomena and paradoxical skin manifestations that may present as the same type of clinical indications for which the TNFi effectively used. Thus, they may display as arthritis, uveitis, colitis, psoriasis, and several other cutaneous clinical manifestations, among them the development of morphea, a localized scleroderma skin lesion. CASE PRESENTATION: We describe a 58-year-old woman with seronegative RA, refractory to methotrexate, who was treated with ABP-501 (Hefiya), an adalimumab (ADA) biosimilar and developed an oval-shaped, deep skin lesion of approximately 3.5cm in size, affecting the left part of her back compatible with morphea 3 months after the initiation of therapy. ADA biosimilar was discontinued and two months later, she had substantial skin improvement. CONCLUSION: This is the first report of morphea manifestation during TNFi biosimilar since the patient had no other trigger factors for morphea development like trauma and infections. Physicians dealing with patients treated with TNFi biosimilars should be aware of paradoxical skin reactions, among them morphea; thus, close monitoring, a minute and careful clinical examination, and a follow- up check are required.


Assuntos
Adalimumab , Antirreumáticos , Medicamentos Biossimilares , Esclerodermia Localizada , Humanos , Feminino , Esclerodermia Localizada/induzido quimicamente , Esclerodermia Localizada/tratamento farmacológico , Pessoa de Meia-Idade , Adalimumab/efeitos adversos , Adalimumab/uso terapêutico , Medicamentos Biossimilares/uso terapêutico , Medicamentos Biossimilares/efeitos adversos , Antirreumáticos/efeitos adversos , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/complicações
20.
Am J Obstet Gynecol MFM ; 6(1): 101226, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37972926

RESUMO

BACKGROUND: Increased rates of adverse pregnancy outcomes have been reported in association with rheumatologic diseases such as systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis. However, little is known about pregnancy outcomes in patients with autoimmune skin diseases. OBJECTIVE: This study aimed to determine the frequency of adverse pregnancy outcomes in patients with autoimmune skin diseases. We hypothesized that similar to rheumatic diseases, the rate of adverse pregnancy outcomes in patients with autoimmune skin diseases would be higher than the general population. STUDY DESIGN: This is a case control study using the TriNetX US Collaborative Network, which is a database of electronic medical records of >95 million patients seen at 57 healthcare organizations in the United States. All pregnant women between the ages of 15 and 44 years who were seen at a healthcare organization between January 1, 2016 and December 31, 2021 were included. Participants with autoimmune skin disease were matched to healthy controls and controls with systemic rheumatologic conditions (systemic lupus erythematosus or rheumatoid arthritis). For both the autoimmune skin disease and healthy control groups, those with systemic rheumatologic condition or hidradenitis suppurativa were excluded. The primary outcomes were adverse pregnancy outcomes defined as spontaneous abortion, gestational hypertension, preeclampsia or eclampsia, gestational diabetes mellitus, intrauterine growth restriction, preterm premature rupture of membranes, preterm birth, and stillbirth. Patients with autoimmune skin diseases and controls were 1:1 propensity score-matched by age, race, ethnicity, comorbidities, obesity, and substance use. For each outcome, odds ratio with a 95% confidence interval was calculated. RESULTS: A total of 2788 patients with autoimmune skin diseases were matched to 2788 healthy controls. Patients with autoimmune skin diseases were at a higher risk of spontaneous abortions than controls (odds ratio, 1.54; 95% confidence interval, 1.36-1.75; P<.001). Compared with patients with systemic lupus erythematosus, patients with autoimmune skin diseases were at lower risk of having infants with intrauterine growth restriction (odds ratio, 0.59; 95% confidence interval, 0.4-0.87; P=.01), preterm birth (odds ratio, 0.68; 95% confidence interval, 0.47-0.98; P=.04), and stillbirth (odds ratio, 0.50; 95% confidence interval, 0.25-0.97; P=.04). The differences in adverse pregnancy outcomes between patients with autoimmune skin diseases and those with rheumatoid arthritis were not statistically significant. CONCLUSION: Patients with autoimmune skin diseases are at a higher risk of spontaneous abortions than patients without autoimmune skin diseases. When analyzed by each autoimmune skin disease, patients with cutaneous lupus erythematosus or vitiligo remained at increased risk of spontaneous abortions compared with patients without autoimmune skin diseases. Patients with autoimmune skin diseases have similar risks of adverse pregnancy outcomes as patients with rheumatoid arthritis, but lower risks than patients with systemic lupus erythematosus.


Assuntos
Aborto Espontâneo , Artrite Reumatoide , Lúpus Eritematoso Sistêmico , Complicações na Gravidez , Nascimento Prematuro , Dermatopatias , Humanos , Recém-Nascido , Gravidez , Feminino , Estados Unidos/epidemiologia , Adolescente , Adulto Jovem , Adulto , Resultado da Gravidez/epidemiologia , Aborto Espontâneo/epidemiologia , Aborto Espontâneo/etiologia , Nascimento Prematuro/epidemiologia , Nascimento Prematuro/etiologia , Natimorto/epidemiologia , Estudos de Casos e Controles , Retardo do Crescimento Fetal/diagnóstico , Retardo do Crescimento Fetal/epidemiologia , Retardo do Crescimento Fetal/etiologia , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/epidemiologia , Complicações na Gravidez/etiologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , Artrite Reumatoide/complicações , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/epidemiologia , Dermatopatias/complicações
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