Localized scleroderma manifesting with skin lesions associated with mechanical stress

2019 ◽  
Vol 29 (4) ◽  
pp. 439-440
Author(s):  
Satoshi Toyama ◽  
Shinichi Sato ◽  
Yoshihide Asano
Keyword(s):  
Mechanical Stress ◽  
Skin Lesions ◽  
Localized Scleroderma

scholarly journals Gender, clinical and topographic features of the localized scleroderma lesions in anogenital area

2020 ◽  
Vol 23 (3) ◽  
pp. 165-173
Author(s):  
Elena S. Snarskaya ◽  
Yulia A. Semenchak
Keyword(s):  
Clinical Symptoms ◽  
Skin Lesions ◽  
Localized Scleroderma ◽  
Steady Increase ◽  
Family Doctors ◽  
Topographic Features ◽  
Anogenital Area ◽  
Clinical Variants ◽  
Limited Scleroderma ◽  
And Gender

Background: Currently, there is an increase in the number of skin lesions of anogenital localization, which is a silent epidemic, both due to the steady increase in the incidence of this pathology, and the interdisciplinary aspect of this problem. Materials and methods: In the article, the authors first analyzed and presented the data of clinical and morphological analysis of 104 patients with various clinical variants of limited scleroderma, on the basis of which the main phenotypic and gender-specific clinical and topographical features of anogenital zone lesions in this group of patients are presented. Results: Scleroatrophic lichen is one of the clinical variants of limited scleroderma, which is characterized by damage to the mucous membranes of the external genitals in both women and men. Lesions of such localization are late and often mistakenly diagnosed by specialists of related disciplines (obstetricians, gynecologists, urologists, family doctors, allergists, cosmetologists), which leads to high risks of developing genitourenal syndrome. Conclusions: The development of scleroatrophic lesions in the anogenital zone is accompanied by pronounced clinical symptoms, including: itching, pain of varying intensity, dysuria, dyspareunia and significant sexual dysfunction.

scholarly journals New Features for Measuring Disease Activity in Pediatric Localized Scleroderma

2018 ◽  
Vol 45 (12) ◽  
pp. 1680-1688 ◽  
Author(s):  
Suzanne C. Li ◽  
Xiaohu Li ◽  
Elena Pope ◽  
Katie Stewart ◽  
Gloria C. Higgins ◽  
...  
Keyword(s):  
Disease Activity ◽  
Age Of Onset ◽  
Skin Lesions ◽  
Inactive Disease ◽  
Localized Scleroderma ◽  
Relative Importance ◽  
Skin Texture ◽  
Disease Patterns ◽  
Disease Extension ◽  
Multicenter Prospective Study

Objective.To identify clinical features that define disease activity in pediatric localized scleroderma (LS), and determine their specificity and importance.Methods.We conducted a multicenter prospective study of patients with active and inactive LS skin lesions. A standardized evaluation of a single designated study lesion per subject was performed at 3 visits. We evaluated the pattern and correlation between assessed features and physician’s global assessments of activity (PGA-A).Results.Ninety of 103 subjects had evaluable data; 66 had active and 24 inactive disease. Subjects had similar age of onset, sex, and disease patterns. Linear scleroderma was the most common subtype. Features specific for active disease included erythema, violaceous color, tactile warmth, abnormal skin texture, and disease extension. Scores for these variables changed over time and correlated with PGA-A of the lesion. Active and inactive lesions could not be distinguished by the presence or level of skin thickening, either of lesion edge or center. However, in active lesions, skin thickening scores did correlate with PGA–A scores. Regression analysis identified the combination of erythema, disease extension, violaceous color, skin thickening, and abnormal texture as predictive of PGA-A at study entry. Damage features were common irrespective of activity status.Conclusion.We identified variables strongly associated with disease activity, expanding upon those used in current measures, and determined their relative importance in physician activity scoring. Skin thickening was found to lack specificity for disease activity. These results will help guide development of a sensitive, responsive activity tool to improve care of patients with LS.

Clinical Manifestations of Scleroderma

1995 ◽  
Vol 16 (2) ◽  
pp. 49-49
Author(s):  
Patricia L. Haber
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Localized Scleroderma ◽  
Unknown Etiology ◽  
Collagen Deposition ◽  
Muscle Fibrosis ◽  
Favorable Prognosis ◽  
Organ Systems

Scleroderma is a connective tissue disease of unknown etiology. Its most characteristic feature is thickening of the skin due to increased collagen deposition. However, the disease may involve multiple other organ systems. Two broad categories of scleroderma have been defined: localized and systemic. Although all forms of scleroderma are rare, localized scleroderma occurs more frequently than systemic sclerosis and has a more favorable prognosis. Several types of localized scleroderma exist. Morphea is characterized by the presence of one or more patches of hard, ivory-colored skin lesions. They begin with erythema and progress to nonpitting edema before becoming sclerotic. The margins of active lesions often have a violaceous hue. Underlying muscle fibrosis and atrophy may occur.

scholarly journals Localized scleroderma: assessment of the therapeutic response to phototherapy

2012 ◽  
Vol 87 (1) ◽  
pp. 63-69 ◽  
Author(s):  
Roberta Buense ◽  
Ida Alzira Gomes Duarte ◽  
Marcio Bouer
Keyword(s):  
Therapeutic Response ◽  
Skin Lesions ◽  
Therapeutic Modality ◽  
Localized Scleroderma ◽  
Treatment Comparison ◽  
Dermal Thickness ◽  
Evolutive Stage ◽  
Skin Ultrasound ◽  
Development Literature ◽  
Chronic Autoimmune Disease

BACKGROUND: Scleroderma is a chronic autoimmune disease characterized by progressive connective tissue sclerosis and microcirculatory changes. Localized scleroderma is considered a limited disease. However, in some cases atrophic and deforming lesions may be observed that hinder the normal development. Literature reports indicate phototherapy as a therapeutic modality with favorable response in cutaneous forms of scleroderma. OBJECTIVES: This study had the purpose of assessing the phototherapy treatment for localized scleroderma. METHODS: Patients with localized scleroderma were selected for phototherapy treatment. They were classified according to the type of localized scleroderma and evolutive stage of the lesions. Clinical examination and skin ultrasound were used to demonstrate the results thus obtained. RESULTS: Some clinical improvement was observed after an average of 10 phototherapeutic sessions. All skin lesions were softer at clinical palpation with scores reduction upon pre and post treatment comparison. The ultrasound showed that most of the assessed lesions presented a decrease in dermal thickness, and only five maintained their previous measure. Treatment response was similar regardless of the type of phototherapeutic treatment employed. CONCLUSIONS: The proposed treatment was effective for all lesions, regardless of the phototherapeutic modality employed. The improvement was observed in all treated skin lesions and confirmed by clinical evaluation and skin ultrasound.

scholarly journals Juvenile Localized Scleroderma. Questions of Treatment

2020 ◽  
Vol 19 (3) ◽  
pp. 228-234
Author(s):  
Rinat K. Raupov ◽  
Mikhail M. Kostik
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Treatment Efficacy ◽  
Active Phase ◽  
Skin Lesions ◽  
Localized Scleroderma ◽  
Resonance Imaging ◽  
Childhood Diseases ◽  
Main Symptom ◽  
Juvenile Localized Scleroderma

Juvenile localized scleroderma (JLS) is a group of childhood diseases with the main symptom — skin and subcutaneous structures lesions, without any organ involvement. There is active (inflammatory) and fibrotic phase in development of JLS. The JLS treatment during active phase (when skin lesions are reversible) is the most effective. The management is determined by the area and depth of skin lesions, appearance and spread of new lesions, presence of extracutaneous signs of the disease. Topical and systemic immunosuppressants are the basic therapy for JLS. The use of antibiotics is not suggested. Clinical scores (LoSCAT), ultrasound, thermography and magnetic resonance imaging are recommended to estimate the treatment efficacy.

scholarly journals Mutation in LEMD3 (Man1) Associated with Osteopoikilosis and Late-Onset Generalized Morphea: A New Buschke-Ollendorf Syndrome Variant

2016 ◽  
Vol 2016 ◽  
pp. 1-9
Author(s):  
Benjamin Korman ◽  
Jun Wei ◽  
Anne Laumann ◽  
Polly Ferguson ◽  
John Varga
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Late Onset ◽  
Skin Lesions ◽  
Localized Scleroderma ◽  
Adult Onset ◽  
Unique Case ◽  
Gene Encoding ◽  
Inner Nuclear Membrane Protein ◽  
Bone Abnormalities

Introduction. Buschke-Ollendorf syndrome (BOS) is an uncommon syndrome characterized by osteopoikilosis and other bone abnormalities, accompanied by skin lesions, most frequently connective tissue nevi. BOS is caused by mutations in theLEMD3gene, which encodes the inner nuclear membrane protein Man1. We describe a unique case of osteopoikilosis associated with late-onset localized scleroderma and familialLEMD3mutations.Case Report. A 72-year-old woman presented with adult-onset diffuse morphea and bullous skin lesions. Evaluation revealed multiple hyperostotic lesions (osteopoikilosis) suggestive of BOS. DNA sequencing identified a previously undescribed nonsense mutation (Trp621X) in theLEMD3gene encoding Man1. Two additional family members were found to have osteopoikilosis and carry the sameLEMD3mutation.Conclusions and Relevance. We report a unique familialLEMD3mutation in an individual with osteopoikilosis and late-onset morphea. We propose that this constellation represents a novel syndromic variant of BOS.

scholarly journals Influence of Childhood Scleroderma on Physical Function and Quality of Life

2010 ◽  
Vol 38 (1) ◽  
pp. 167-173 ◽  
Author(s):  
EILEEN M. BAILDAM ◽  
HOLLY ENNIS ◽  
HELEN E. FOSTER ◽  
LINDSAY SHAW ◽  
ALICE S.E. CHIENG ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Child Health ◽  
Physical Function ◽  
Demographic Data ◽  
Life Quality ◽  
Skin Lesions ◽  
Localized Scleroderma ◽  
Life Questionnaire ◽  
Cross Sectional

Objective.There have been few studies of quality of life in childhood scleroderma and these focused predominantly on self-perception and the influence of skin lesions. Our cross-sectional study aimed to describe the influence of childhood scleroderma on physical function and quality of life in relation to clinical and demographic measures.Methods.Children with either localized scleroderma or systemic sclerosis (SSc) attending pediatric rheumatology clinics, together with their parents or guardians, were asked to complete a set of 4 validated measures. Clinical and demographic data were provided by consultant pediatric rheumatologists.Results.In total, 28 children and their parents/guardians participated in the study (68% female, median age 13 yrs; 86% localized scleroderma, 14% SSc). The median Child Health Assessment Questionnaire (CHAQ) score was 0.1 (range 0–3, 0 indicating no impairment), the median Child Dermatology Life Quality Index (CDLQI) score was 5 (range 0–30, 0 indicating no impairment), and the median Child Quality of Life Questionnaire (CQOL) function score was 26 (range 0–105, 0 indicating no impairment). Family activity, measured by the Child Health Questionnaire (CHQ-PF50), was also moderately impaired by scleroderma, with a median score of 83 (0–100, 100 indicating no impairment).Conclusion.Scleroderma had only a moderate effect on quality of life and physical function as measured by the 4 validated instruments. Although a small number of children reported greater impairment, this is an encouraging finding, given its potential disfiguring and debilitating effects.

scholarly journals Initial evaluation of an ultrasound measure for assessing the activity of skin lesions in juvenile localized scleroderma

2011 ◽  
Vol 63 (5) ◽  
pp. 735-742 ◽  
Author(s):  
S. C. Li ◽  
M. S. Liebling ◽  
K. A. Haines ◽  
J. E. Weiss ◽  
A. Prann
Keyword(s):  
Skin Lesions ◽  
Initial Evaluation ◽  
Localized Scleroderma ◽  
Juvenile Localized Scleroderma
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