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Localized scleroderma designates a heterogeneous group of sclerotic skin disorders. Depending on the subtype, severity, and site affected, adjacent structures such as adipose tissue, muscles, joints, and bones may be involved. This is an update of the existing German AWMF (Association of the Scientific Medical Societies in Germany) guidelines (clas...
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... systemic sclerosis, whose diffuse (anti-Scl-70 or antitopoisomerase-1 antibodies) and limited (anti-centromere antibodies) forms are frequently characterized by highly specific antibodies, there are no characteristic serologic parameters in LS. In the authors' opinion, apart from basic laboratory tests (CBC with differential, clinical chemistry, antinuclear antibodies), special lab tests should only be performed if systemic sclerosis is suspected or to be ruled out (in this case including anti-Scl-70 or anti-centromere antibodies) ( Table 2). In case of eosinophilic fasciitis, electrophoresis and quantitative measurement of immunoglobulins should be part of the workup. ...Context 2
... In all forms of localized scleroderma, blood should be drawn for basic laboratory tests (CBC with differential and clinical chemistry) and antinuclear antibodies ( Table 2). ...Context 3
... is therefore, in the authors' opinion, the treatment of first choice in limited LS. By contrast, UV radiation is not suitable for variants that involve deeper structures (fat, fascia, muscle, bone) (Table 2). Below, study results of various treatment modalities are summarized and evaluated. ...Context 4
... systemic sclerosis, whose diffuse (anti-Scl-70 or antitopoisomerase-1 antibodies) and limited (anti-centromere antibodies) forms are frequently characterized by highly specific antibodies, there are no characteristic serologic parameters in LS. In the authors' opinion, apart from basic laboratory tests (CBC with differential, clinical chemistry, antinuclear antibodies), special lab tests should only be performed if systemic sclerosis is suspected or to be ruled out (in this case including anti-Scl-70 or anti-centromere antibodies) ( Table 2). In case of eosinophilic fasciitis, electrophoresis and quantitative measurement of immunoglobulins should be part of the workup. ...Context 5
... In all forms of localized scleroderma, blood should be drawn for basic laboratory tests (CBC with differential and clinical chemistry) and antinuclear antibodies ( Table 2). ...Context 6
... is therefore, in the authors' opinion, the treatment of first choice in limited LS. By contrast, UV radiation is not suitable for variants that involve deeper structures (fat, fascia, muscle, bone) (Table 2). Below, study results of various treatment modalities are summarized and evaluated. ...Citations
... Topical corticosteroids (highly potent: once a day for up to 4 weeks, moderately potent: once a day for up to 3 months) are effective for subtypes with limited skin involvement (affecting the dermis). Methotrexate (adults: 12.5-25 mg every week, children: 15 mg/m 2 every week, max: 25 mg every week) or systemic corticosteroids (adults: methylprednisolone 500-1,000 mg IV daily on three consecutive days every month for at least 3-6 months, children: methylprednisolone 30 mg/kg IV daily (maximum 1,000 mg) on three consecutive days every month for at least 3-6 months) are useful for subtypes with severe skin and/or musculoskeletal involvement (affecting the adipose tissue, fasciae, muscles, joints, and bones or extensive skin involvement) (12). In the present case, treatment started with EBETREX Tablet 2.5 mg for 3 years when she was 13 years old and the lesion was controlled; therefore, there was no pale linear region on her face. ...
Background: In this study, the clinical and radiographic manifestations of a patient with linear morphea as well as her clinical features are reported with more emphasis on the oral effects of this connective tissue disease. This patient was referred to the orthodontic department due to the misalignment of her teeth and a diagnosis of linear scleroderma made three years earlier by her dermatologist. The most important intervention in such patients is to not start treatment without knowledge, since it will cause more harm to the patient. Therefore, it is necessary to find the best treatment plan with the fewest complications as soon as possible.
... Original Research встречаются линейные формы, для которых характерно агрессивное течение с вовлечением глубоких тканей, вплоть до развития деструкции и деформации костей. К таким тяжелым формам относятся: склеродермия лица по типу «удар саблей», для которой характерно быстрое развитие атрофии костной ткани черепа с формированием тяжелых косметических дефектов; склеродермия, протекающая с преимущественной локализацией очагов на конечностях, которые часто приводят к развитию сухожильно-мышечных контрактур и нарушению роста конечностей, что является частой причиной инвалидизации [9,10,12]. Клиническое течение ограниченной склеродермии в детском возрасте, в отличие от взрослых, имеет свои особенности, в связи с чем изучение особенностей клинического течения ограниченной склеродермии у детей с учетом их возраста позволяет выявить механизмы формирования болезни в каждом конкретном возрастном периоде, оценить роль иммунитета как фактора развития. Изучение роли этих факторов в манифестации и формировании ОС у детей дает возможность понять патогенетическую природу болезни [13][14][15]. ...
Введение. Представлены данные клинического исследования, описывающие особенности клинических проявлений ограниченной склеродермии у детей. Описаны дерматоскопические симптомы на разных стадиях развития заболевания и частота встречаемости отдельных признаков. Цель. Изучить частоту встречаемости и особенности клинических и дерматоскопических симптомов при ограниченной склеродермии у детей. Материалы и методы. Проведен клинический и дерматоскопический анализ 68 пациентов с ограниченной склеродермией. Результаты. Ограниченная склеродермия в большинстве случаев встречается у девочек (1:2,1), средний возраст пациентов составил 5,9±0,4 года. Клиническая картина характеризуется наличием в 88,2% случаев множества очагов (2–6 очагов, среднее количество очагов – 2,6±0,23) с преобладанием линейных и бляшечных форм (57,4% и 39,7% соответственно). Особенности дерматоскопических признаков ограниченной склеродермии зависели от стадии заболевания.
Introduction. The data of a clinical study describing the features of the clinical manifestations of localized scleroderma in children are presented. Dermoscopic symptoms at different stages of the development of the disease and the frequency of occurrence of individual signs are described. Purpose. To study the frequency of occurrence and features of clinical and dermoscopic symptoms in localized scleroderma in children. Materials and methods. Clinical and dermatoscopic analysis of 68 sick children with localized scleroderma was carried out. Results. LS in children in most cases occurs in girls (1:2.1), the average age of patients was 5.9±0.4. The clinical picture is characterized by the presence in 88.2% of cases of multiple foci (2–6 foci, the average number of foci is 2.6±0.23) with a predominance of linear and plaque forms (57.4% and 39.7%, respectively). Features of dermatoscopic signs of localized scleroderma depended on the stage of the disease.
... As summarised in Fig. 1, 1233 unique references were identified by database searches, 720 of which were excluded during title and abstract screening as they were not related to the subject of this review. Of the remaining full-text 513 articles, 109 were identified as meeting the inclusion criteria: 3 guidelines and treatment recommendations [1,9,10] (Table 1); 4 systematic reviews and meta-analyses [11][12][13][14] ( Table 2); 42 primary studies including 9 prospective studies [15][16][17][18][19][20][21][22][23] (Table 3) and 33 retrospective observational studies [4, (Table 4 [99]; and 16 overviews and expert opinions [5,7,[100][101][102][103][104][105][106][107][108][109][110][111][112][113] ( Table 6). ...
... Knobler et al. [108] Expert opinion: Guideline European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes. Kreuter et al. [109] Expert opinion: Guideline German guidelines for the diagnosis and therapy of localized scleroderma. Li et al. [5] Clinician survey Treatment of pediatric localized scleroderma: results of a survey of North American pediatric rheumatologists. ...
Juvenile localised scleroderma (JLS) is a condition that results in inflammation and fibrosis of the skin in children and young people. Systemic treatment with immunomodulation is most commonly with Methotrexate (MTX) or Mycophenolate Mofetil (MMF). Other treatments include DMARDs, biologic therapies, topical treatments and phototherapy. This scoping review considers the available information on the relative safety and efficacy of MTX and MMF. A scoping review was conducted in accordance with PRISMA-ScR guidelines. A search was conducted in three bibliographic databases (Cochrane Library, Medline (OVID) and Embase (OVID)) to identify relevant studies for inclusion . A single reviewer identified published articles eligible for the review based on the inclusion and exclusion criteria. The relevant key findings were summarised in a word document by the first reviewer and then checked by a second reviewer. From 1233 unique references, 109 were identified as meeting the inclusion criteria. MTX is the most commonly used first-line systemic treatment for JLS with the greatest evidence for its use in JLS. The evidence for the efficacy of MMF is restricted to a small number of retrospective studies. Both MTX and MMF are described to be relatively safe medications with a low rate of adverse events. Information regarding the tolerability of these medications is limited. The rarity of JLS and the paucity of validated measures of disease activity makes comparison between these two treatments challenging and should be reflected in the design of future studies.
... 5 Kreuter et al. consider that, depending on the subtype, morphea can also involve adjacent tissues such as the fat, fascia, muscle, and bone, but not internal organs. 2 Thus, screening for systemic damage in patients with morphea is not a common practice. Moreover, several authors have investigated the prevalence of systemic disease in patients with morphea and found that signs indicative of SSc are statistically not more frequent in patients with morphea than in controls, which supports the view that morphea and SSc are not part of a single disease continuum. ...
Morphea is an auto‐immune disease, and its association with other immune‐mediated diseases should not come as a surprise. Dermatologists should be aware of its possible coexistence with severe systemic involvement. image
... LSA and morphea occasionally coexist. 2 Both BLS and generalized morphea usually develop chronically and respond poorly to treatment. ...
... 3 The patient described here had extragenital bullous ulcers and extensive body sclerotic plaques, which have only rarely been reported.There is no effective therapeutic regimen for either BLS or generalized morphea. Corticosteroids, methotrexate, and phototherapy are the main therapies at present.1,2 The treatment experience of LSA all comes from case report.1 ...
We here report a case of a middle-aged man with an unusual case of bullous lichen sclerosus complicated with generalized morphea. He showed initial recurrent flaccid bullae, followed by ivory-white sclerotic plaques and extensive skin sclerosis, with additional walking disorder caused by knee-joint contracture and ulcers on the lower extremities and back. The patient had no visceral involvement. After oral hydroxychloroquine and oral corticosteroids failed, the patient was given tofacitinib, which resolved his ulcers after 4 weeks and ameliorated his knee-joint contracture and skin sclerosis within 4 months. Owing to the occurrence of diffuse large B-cell lymphoma, he stopped using tofacitinib, and the ulcer and walking disorder reappeared. This is rare case of bullous lichen sclerosus-generalized morphea overlap syndrome. The patient recovered well after treatment with tofacitinib. His symptoms recurred after discontinuation of tofacitinib.
... However, UVA1/ PUVA has limited indications, and poses a significantly higher risk of skin cancer, especially squamous cell carcinoma and melanoma. NB-UVB has also been demonstrated to penetrate deep into the dermis as opposed to broad-band UVB, which further encourages us to choose this treatment [9]. ...
... Wykazano także, że NB-UVB przenika głęboko do skóry właściwej w przeciwieństwie do fototerapii UVB o szerokim zakresie. Stanowi to dodatkowy czynnik przemawiający za wyborem NB-UVB w leczeniu [9]. ...
... ŭ žƈƋƅƟžƀſƇƇƟ, ƉƊƈżſžſƇƈƆƍ ż ŚƇŽƅƟƠ ƌź őƊƅźƇ-žƟƠ, ƑźƋƌƈƌź żƂƇƂƄƇſƇƇƙ ƁźƏżƈƊƘżźƇƇƙ ƋƌźƇƈ-żƂƅź 3,4 żƂƉźžƄƍ Ƈź 1 ƆƅƇ žƟƌſƃ. ŧźƃƑźƋƌƟƒſ ŸūŞ žſŻƘƌƍƝ žƈ ƉƍŻſƊƌźƌƇƈŽƈ ƋƌƊƂŻƄź ƁƊƈ-ƋƌźƇƇƙ (8)(9)(10)(11)(12)(13) ƊƈƄƟż -ƍ žƟżƑźƌƈƄ, 10-15 Ɗƈ-ƄƟż -ƍ ƏƅƈƉƑƂƄƟż) [5]. ...
Локалізована склеродермія характеризується появою вогнищ атрофії шкіри та підшкірних тканин, але може спостерігатися ураження глибоких м’яких тканин, кісток і суглобів, що, крім косметичних змін, може призвести до функціональних порушень і болю. Проведено зіставлення даних літератури та особистих спостережень про особливості дебюту, розгортання клінічної симптоматики, перебігу, результатів лабораторного та інструментального обстеження в дітей з локалізованою склеродермією.
Мета – проаналізувати власні дані діагностики та перебігу патологічного процесу у хворих із локалізованою формою ювенільної склеродермії; зіставити власні спостереження та дані літературних джерел щодо діагностики цієї хвороби.
Матеріали та методи. За період 2010-2020 рр. під нашим наглядом перебувало 48 дітей з ювенільною склеродермією. Пацієнтам проведено ультразвукове дослідження судин, органів черевної порожнини, серця та суглобів, рентгенологічне дослідження легень і суглобів, електрокардіографічне дослідження, магнітно-резонансну томографію, визначено показники аутоімунної активності.
Результати. Проаналізовано особливості перебігу та діагностики ювенільної локалізованої склеродермії, зокрема, ураження шкіри спостерігається у вигляді запальних осередків (еритеми), далі спостерігається розвиток осередків склерозу та атрофії. Вогнища можуть бути одиничними або множинними. У всіх хворих виявляється синдром Рейно, у більшості випадків - суглобовий синдром у вигляді артралгій. Під час лабораторних досліджень показники загальної запальної активності при локалізованій склеродермії не завжди є інформативними. Ультразвукове сканування шкіри та м’язів (інноваційне обстеження, яке на сучасному етапі впроваджується як конкуренція травматичної біопсії) є інформативним методом діагностики змін дерми, підшкірної клітковини, м’яких тканин і судин, що є ознакою активної склеродермії.
Висновки. Локалізована ювенільна склеродермія має певні особливості клінічного перебігу, що треба мати на увазі при встановленні діагнозу: локальне ураження шкіри у вигляді запальних осередків (еритеми), далі спостерігається розвиток осередків склерозу та атрофії, синдром Рейно, суглобовий синдром у вигляді артралгій, неінформативні лабораторні методи дослідження, вісцеральні ураження найчастіше проявляються функціональними розладами, нерівномірність патологічного процесу в судинному руслі зі змінами насамперед комплексу інтима-медіа, інформативне ультразвукове сканування шкіри та м’язів.
Дослідження виконано відповідно до принципів Гельсінської декларації. Протокол дослідження ухвалено Локальним етичним комітетом зазначеної в роботі установи. На проведення досліджень отримано інформовану згоду батьків дітей.
Автори заявляють про відсутність конфлікту інтересів.
... As to the treatment, some patients who have intractable epilepsy are given a combined medication or even surgical removal of the lesion. Corticosteroids and methotrexate are beneficial to control the development of seizures and skin sclerosis [19]. The use of methylprednisolone in our case had achieved a great effect. ...
Linear scleroderma is the most common type of localized scleroderma in children. Lesions rarely involve areas other than the skin, and nervous system involvement is even rare. We reported a case of a 6-year-old girl who was admitted to the hospital with recurrent seizures for 4 weeks. Before that, she had left frontal plaques for more than 1 year. Radiological imaging of the brain showed multiple abnormal lesions and skin biopsy of the plaques indicated scleroderma. After drug therapy, the girl had no recurrence of epilepsy, and no obvious abnormalities were found in the reexamination of neuroimaging. We performed further radiological examination on this patient and reviewed the literatures for this rare case.
... Most widely used for this purpose are topical corticosteroids, tacrolimus ointment, calcipotriol ointment, ultraviolet phototherapy, systemic corticosteroids, oral methotrexate, mycophenolate mofetil, and biological agent. [3][4][5] However, in a setting of disease progression and recurrence, attempting other therapeutic regimens is warranted. ...
Linear scleroderma en coup de sabre (LSCS) is a variant of localized scleroderma associated with band-like fibrotic lesions in the frontoparietal area. We report a case of LSCS in a woman who presented with progressive mild hyperchromia on the right side of her forehead, with dermal atrophy and hair and eyebrow loss. After the failure of conservative treatments, the patient responded dramatically to injection of autologous localized concentrated growth factor. After three treatments, the atrophy, stiffness, and angiotelectasis on the affected area had improved. No recurrence was detected 24 months after the last treatment. This is the first study describing the use of autologous concentrated growth factor injection to alleviate clinical symptoms of LSCS. This suggests that concentrated growth factor may be a treatment for LSCS in the clinic.
... 3 Among the rarely reported morphea precipitating factors are surgical procedures 4 such as mastectomies and the insertion of silicone breast implants. 3,[5][6][7] In most of these reports, the authors have considered radiotherapy after the mastectomy or a foreign body reaction to the implant as the triggering factor without attributing a role to the trauma of surgery. However, a recent study reported that 16% of morphea lesions appeared at sites of previous skin trauma, 8 which could be associated with the development of morphea after surgical procedures. ...
Liposuction is a common aesthetic procedure; however, to date, liposuction has not been linked to morphea. The aim was to review cases with a history of liposuction that presented active morphea lesions in the same surgery regions and were confirmed by ultrasound and histology. A retrospective descriptive analysis of the clinical, ultrasonographic, and pathology database took place (2014–2020). Eleven patients met the criteria. Ultrasound supported the diagnosis, and the ultrasonographic signs of activity in these cases matched the features described in the literature in 100% of cases. In summary, morphea may appear after liposuction and ultrasound can support its early detection.
