Systemic involvement in localized scleroderma/morphea

Górkiewicz-Petkow, Anna; Kalińska-Bienias, Agnieszka

doi:10.1016/j.clindermatol.2015.05.009

Cited by 12 publications

(9 citation statements)

References 42 publications

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“…Extracutaneous involvement such as musculoskeletal (arthritis, arthralgia), neurological, ocular problems, respiratory (coughing, dyspnea, restrictive lung disease), gastrointestinal tract (gastroesophageal reflux, esophagitis), cardiac, kidney disorders and Raynaud phenomenon can be seen in morphea patients [ 9 ]. Systemic involvement has been reported to occur in 24–46% of generalized and linear morphea cases.…”

Section: Discussionmentioning

confidence: 99%

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Morphea in Middle Anatolia, Turkey: a 5-year single-center experience

Bulur¹,

Erdoğan²,

Karapınar³

et al. 2017

pdia

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IntroductionMorphea, also referred to as localized scleroderma, is a rare fibrosing skin disorder of undetermined cause.AimWe report our single-center experience with morphea.Material and methodsThe study included 53 patients who were diagnosed with morphea by histopathology in our department between 2010 and 2015. Study data were collected retrospectively from the records of morphea patients.ResultsThe study included 53 patients (38 women, 15 men), and median age at onset was 39.0 (range: 8–85) years. Thirty (56.6%) patients had circumscribed morphea, 15 (28.3%) had generalized morphea, and 7 (13.2%) had linear morphea. One patient had mixed variant morphea (generalized, pansclerotic and linear morphea). ANA positivity was detected in 12 (22.6%) patients, but analysis for an association between the presence of ANA and morphea types, patients’ characteristics did not reveal any significant associations. We did not observe any extracutaneous manifestations in patients during follow-up period. There were 2 of 53 patients who had concomitant autoimmune disorder including vitiligo and spondyloarthritis. Thirty (56.6%) patients received only topical treatment. The patients with clinical improvement who were treated with systemic therapy received methotrexate (26.4%), colchicine (9.4%), mycophenolate mofetil (5.7%) and prednisolone (1.9%).ConclusionsOur results related to the demographic data of the patients and morphea types were consistent with the literature. On the other hand we observed that methotrexate was mostly used as an effective treatment option for generalized morphea.

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Section: Discussionmentioning

confidence: 99%

“…The incidence of ANA positivity in morphea cases has been reported as 5.9–73% [ 8 ]. ANA positivity has been reported to be more common in adults (45–53%) than in children (26–53%) with morphea [ 8 , 9 ]. Besides, the ANA positivity rate has been reported to be higher in generalized morphea [ 5 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

Morphea in Middle Anatolia, Turkey: a 5-year single-center experience

Bulur¹,

Erdoğan²,

Karapınar³

et al. 2017

pdia

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“…LoS and SSc cannot be differentiated by histopathological examination because they share the same aspects: lymphocytic perivascular infiltration in the reticular dermis and swollen endothelial cells in the early phase, followed by thickened collagen bundles infiltrating the entire dermis and extending into subcutaneous fat in the late phase, with loss of eccrine glands and blood vessels, and “fat trapping.” Therefore, skin biopsy does not allow making differential diagnosis per se; conversely, the global evaluation of the clinical picture is fundamental for the diagnosis. LoS is characterized by the absence of sclerodactyly, RP, and nailfold capillary changes; moreover, even if patients with LoS commonly have nonspecific systemic symptoms, such as malaise, fatigue, arthralgias, and myalgias, as well as the presence of autoantibodies, the typical features of SSc visceral involvement are absent [ 3 , 12 ].…”

Section: Discussionmentioning

confidence: 99%

From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution

Giuggioli

Colaci

Cocchiara

et al. 2018

Dermatology Research and Practice

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Background Systemic sclerosis (SSc) and localized scleroderma (LoS) are two different diseases that may share some features. We evaluated the relationship between SSc and LoS in our case series of SSc patients. Methods We analysed the clinical records of 330 SSc patients, in order to find the eventual occurrence of both the two diseases. Results Eight (2.4%) female patients presented both the two diagnoses in their clinical histories. Six developed LoS prior to SSc; in 4/6 cases, the presence of autoantibodies was observed before SSc diagnosis. Overall, the median time interval between LoS and SSc diagnosis was 18 (range 0–156) months. Conclusions LoS and SSc are two distinct clinical entities that may coexist. Moreover, as anecdotally reported in pediatric populations, we suggested the possible development of SSc in adult patients with LoS, particularly in presence of Raynaud's phenomenon or antinuclear antibodies before the SSc onset.

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“…Inflammatory cells increase the production of profibrotic cytokines. 1 , 3 , 10 , 11 There have been studies that investigated on the role of several cytokines and mediators in the pathogenesis of scleroderma. One of these is transforming growth factor-beta (TGF-β), which plays an important role in tissue fibrosis by stimulating fibroblasts to increase the production of extracellular matrix proteins.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of mean platelet volume in localized scleroderma

Bahalı

Emiroğlu

et al. 2017

An. Bras. Dermatol.

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BackgroundLocalized scleroderma is a chronic inflammatory skin disease characterized by sclerosis of the dermis and subcutaneous tissue. Platelets play an important role in inflammation. Following activation, platelets rapidly release numerous mediators and cytokines, which contribute to inflammation.ObjectivesTo evaluate whether there was any relation between localized scleroderma and platelet parameters.MethodsForty-one patients with localized scleroderma were enrolled in the study. The control group consisted of 30 healthy subjects.ResultsThe mean platelet volume level in the patient group was 9.9 ± 1.3 fl and in the control group was 7.6 ± 1.1 fl. This difference was statistically significant (p< 0.001). The plateletcrit values are minimally higher in the patient group as compared to the control group. It was statistically significant (p<0.001). There was no significant difference in the platelet counts between the two groups (p= 0.560) In the patient group, there was no significant relation between the mean platelet volume levels and clinical signs of disease (p=0.09). However, plateletcrit values are higher in generalized than localized forms of disease (p=0.01).Study LimitationsThe limited number of patients and the retrospective nature of the study were our limitations.ConclusionsThis study suggests that platelets might play a role in the pathogenesis of scleroderma. Platelet parameters may be used as markers for evaluating disease severity and inflammatory processes. Thus, there is a need for more detailed and prospective studies.

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Systemic involvement in localized scleroderma/morphea

Cited by 12 publications

References 42 publications

Morphea in Middle Anatolia, Turkey: a 5-year single-center experience

Morphea in Middle Anatolia, Turkey: a 5-year single-center experience

From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution

Evaluation of mean platelet volume in localized scleroderma

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