Localized Scleroderma: MR Findings and Clinical Features

Schanz, Stefan; Fierlbeck, Gerhard; Ulmer, Anja; Schmalzing, Marc; Kümmerle-Deschner, Jasmin; Claussen, Claus D.; Horger, Marius

doi:10.1148/radiol.11102136

Cited by 76 publications

(44 citation statements)

References 25 publications

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“…A review of MRI findings of LS has shown that fascial thickening and enhancement were notable in addition to subcutaneous septal thickening [13]. In our case, contractured fasciae at the posteromedial side of the thigh could distort the fractured femur as if a strained string had bent a bow.…”

Section: Discussionmentioning

confidence: 69%

Extensive Bone Lengthening for a Patient with Linear Morphea

Mishima

Kitoh

Matsushita

et al. 2018

Case Reports in Orthopedics

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Localized scleroderma, also known as morphea, is a rare condition characterized by progressive sclerosis of the skin and associated atrophy of the underlying tissues. The linear type of localized scleroderma is the most frequent form in childhood, usually affecting unilateral extremities. Fibrosclerosis of the fasciae and muscles can spread across joints and impair the range of motion of the joint. Dysplastic and/or atrophic bones of the affected lower extremity can lead to clinically significant leg length discrepancy (LLD). Limb reconstruction surgery has rarely been indicated for LLD in patients with linear morphea. We report on a case of extensive bone lengthening for appreciable LLD in a pediatric patient with linear morphea. A Japanese girl with linear morphea underwent staged simultaneous lengthening of the femur and tibia twice at seven and eleven years of age using a unilateral external fixator. A healing index exceeded 100 days/cm except for the first femoral lengthening that was complicated by regenerate fracture. At the final follow-up, LLD of 38 mm remained, but she could walk independently without a brace or a crutch. Due to soft tissue tightness and poor regenerative ability in the affected limb, cautions should be taken to prevent regenerate fracture and/or malalignment of the limb.

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Section: Discussionmentioning

confidence: 69%

Extensive Bone Lengthening for a Patient with Linear Morphea

Mishima

Kitoh

Matsushita

et al. 2018

Case Reports in Orthopedics

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“…Due to its depth of penetration, which is a function of frequency, the usefulness of ultrasonography is somewhat limited to the skin and subcutaneous tissue. In this regard MRI is more advantageous and allows for better assessment of deeper structures such as the fascia and underlying muscle [45, 46]. As with ultrasonography, MRI can be resourceful in monitoring disease activity and response to treatment [47].…”

Section: Discussionmentioning

confidence: 99%

Updates on Morphea: Role of Vascular Injury and Advances in Treatment

Sartori‐Valinotti

Tollefson

Reed

2013

Autoimmune Diseases

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Morphea and systemic sclerosis are fibrosing disorders of the skin that share common inflammatory and immunologic pathways that are responsible for the vascular changes, increased collagen production, and extracellular matrix proliferation seen in both conditions. Recent advances in molecular biology techniques have furthered our knowledge of the potential underlying pathogenic mechanisms and offer new and provocative areas of research for novel diagnostic and therapeutic interventions. This review focuses on the role of vascular injury in the development of morphea, the use of ultrasonography as a diagnostic modality, and well-established and newly proposed treatments.

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“…A retrospective study reported MRI findings in 43 morphea patients; MRI enabled confirmation of musculoskeletal abnormalities in all but one of the patients in whom musculoskeletal involvement was clinically suspected [131]. A recent retrospective study confirmed the potential of MRI in detection of musculoskeletal involvement [132].…”

Section: Assessment Of Musculoskeletal Involvementmentioning

confidence: 99%

Morphea and Eosinophilic Fasciitis: An Update

et al. 2017

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Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated. However, extensive extracellular matrix formation and autoimmune dysfunction are thought to be key pathogenic processes. Likewise, these processes are considered essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in clinical presentation between morphea and SSc have led to many theories about their relatedness. Importantly, morphea may be differentiated from SSc based on absence of sclerodactyly, Raynaud’s phenomenon, and nailfold capillary changes. The diagnosis of morphea is often based on characteristic clinical findings. Histopathological evaluation of skin biopsies and laboratory tests are not necessary in the majority of morphea cases. However, full-thickness skin biopsies, containing fascia and muscle tissue, are required for the diagnosis of EF. Monitoring of disease activity and damage, especially of subcutaneous involvement, is one of the most challenging aspects of morphea care. Therefore, data harmonization is crucial for optimizing standard care and for comparability of study results. Recently, the localized scleroderma cutaneous assessment tool (LoSCAT) has been developed and validated for morphea. The LoSCAT is currently the most widely reported outcome measure for morphea. Care providers should take disease subtype, degree of activity, depth of involvement, and quality-of-life impairments into account when initiating treatment. In most patients with circumscribed superficial subtypes, treatment with topical therapies suffices. In more widespread disease, UVA1 phototherapy or systemic treatment with methotrexate (MTX), with or without a systemic corticosteroid combination, should be initiated. Disappointingly, few alternatives for MTX have been described and additional research is still needed to optimize treatment for these debilitating conditions. In this review, we present a state-of-the-art flow chart that guides care providers in the treatment of morphea and EF.

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Localized Scleroderma: MR Findings and Clinical Features

Abstract: MR imaging reveals musculoskeletal involvement in patients with localized scleroderma.

Cited by 76 publications

References 25 publications

Extensive Bone Lengthening for a Patient with Linear Morphea

Extensive Bone Lengthening for a Patient with Linear Morphea

Updates on Morphea: Role of Vascular Injury and Advances in Treatment

Morphea and Eosinophilic Fasciitis: An Update

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