Congenital localized scleroderma

Zulian, Francesco; Vallongo, Cristina; Oliveira, Sheila Knupp Feitosa de; Punaro, Marilynn; Ros, J; Mazur-Zielińska, Henryka; Galéa, P; Dalt, Liviana Da; Eichenfield, Lawrence F.

doi:10.1016/j.jpeds.2006.04.052

Cited by 51 publications

(30 citation statements)

References 12 publications

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“…There are some special cases. There was one female diagnosed in the first week of life, also called "congenital” LS [26]. Her mother had a recent diagnosis of systemic lupus erythematosus.…”

Section: Resultsmentioning

confidence: 99%

A cross-sectional electromyography assessment in linear scleroderma patients

2014

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BackgroundMuscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcutaneous and muscle atrophy. The aim was investigate nerve conduction and muscle involvement by electromyography in pediatric patients with LS.MethodsWe conducted a retrospective review of LS pediatric patients who had regular follow up at a single pediatric center from 1997–2013. We selected participants if they had consistently good follow up and enrolled consecutive patients in the study. We examined LS photos as well as clinical, serological and imaging findings. Electromyograms (EMG) were performed with bilateral symmetric technique, using surface and needle electrodes, comparing the affected side with the contralateral side. Abnormal muscle activity was categorized as a myopathic or neurogenic pattern.ResultsNine LS subjects were selected for EMG, 2 with Parry-Romberg/Hemifacial Atrophy Syndrome, 7 linear scleroderma of an extremity and 2 with mixed forms (linear and morphea). Electromyogram analysis indicated that all but one had asymmetric myopathic pattern in muscles underlying the linear streaks. Motor and sensory nerve conduction was also evaluated in upper and lower limbs and one presented a neurogenic pattern. Masticatory muscle testing showed a myopathic pattern in the atrophic face of 2 cases with head and face involvement.ConclusionIn our small series of LS patients, we found a surprising amount of muscle dysfunction by EMG. The muscle involvement may be possibly related to a secondary peripheral nerve involvement due to LS inflammation and fibrosis. Further collaborative studies to confirm these findings are needed.

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Section: Resultsmentioning

confidence: 99%

A cross-sectional electromyography assessment in linear scleroderma patients

2014

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“…Interestingly, a recent study described six patients with congenital localized scleroderma (CLS) representing less than 1% of all the cases of JLS. The linear subtype is the only manifestation of the disease at this age and, due to its unusual onset, it is often misdiagnosed as skin infection, naevus or salmon patch leading to a diagnostic delay of 2 to 4 years [24 ]. In this view, CLS should be included in the differential diagnosis of neonates and infants with hyperaemic-fibrotic skin lesions in order to avoid functional and aesthetic sequelae and to allow a prompt therapeutic approach.…”

Section: Epidemiologymentioning

confidence: 99%

Localized scleroderma

Laxer

Zulian

2006

Current Opinion in Rheumatology

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“…In one report, the authors note that the initial appearance of morphea may be misdiagnosed as PWS. 17 In another case, en coup de sabre morphea was initially diagnosed as a PWS and treated with the pulsed dye laser. 18 Other skin disorders have been associated with morphea.…”

Section: Discussionmentioning

confidence: 99%