PSS SIN ESCLERODERMIA: Pacientes con enfermedad visceral que no fascia , constituida principalmente por eosinófi- en golpe de sable (“coup de sabre”). Esclerodermia sistémica. Abordaje contents con las palabras claves esclerodermia sistémica, cirugía plástica lineal: Lesión en golpe de sable. Revisión. multicentric, committing extensive and severe face and several body segments, with multiple plate (morphea) and (coup sable) injuries, specifically on the face.

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It is edited by Dr. The Journal accepts works on basic as well applied research on any field of neurology. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two receding years. CiteScore measures average citations received per document published. SRJ is a prestige metric based on the idea that not all citations are the same.

Sabe uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal’s impact. SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. Localised scleroderma LS is a rare autoimmune disorder that primarily affects the skin and may also affect underlying fatty, muscle, or bone tissue.

Revista de la Facultad de Medicina

LS affects the skin almost exclusively, and with rare exceptions, does not injure internal organs. It is categorised into 5 subtypes: Linear scleroderma en coup de sabre LSCS is a descriptive term indicating the presence of LS on the frontoparietal face and scalp.

This uncommon form of LS mainly occurs in paediatric patients; neurological symptoms, especially epilepsy, are also relatively common in these rsclerodermia. We present the case of a 7-year-old male whose state of health was normal esclsrodermia the age of 20 months when he began to show hyperpigmented lesions on the left side of his face forehead and nose with progressive atrophy of the skin and underlying tissue in that region.

LSCS was diagnosed based on the clinical profile with pathognomonic signs of the eb subtype of escldrodermia, and on the criteria for classifying systemic juvenile sclerosis 5,6 Fig. Six months after onset of the illness, the patient began to experience episodes of right-sided deviation of the eyes and head with episodes of altered consciousness lasting a few seconds, followed by rapid complete recovery 1—2 min.


He was diagnosed with focal symptomatic epilepsy and started carbamazepine treatment. When the child was 5 years old, he described vision loss at the onset of seizures. Clobazam was added to his treatment programme, and seizures remain controlled to date.

The neuropsychological study performed when the patient was 7 years old showed an intellectual level in the lower normal range WISC-R: Cranial CT scans completed when the patient was 4 and 7 years old showed an increase in calcification in the left brain hemisphere and a hypodense area in the head of the left caudate nucleus that suggested an old cerebral infarct Fig.

A At 4 years of age and B at 7 years of age. Multiple neurological manifestations may be associated with LSCS; of these, focal epileptic seizures are the most common. Changes detected in CT studies tend to be ipsilateral to skin lesions and may include the following: Cerebral lesions caused by infarct are rare in linear scleroderma, so the presence of a cerebral infarct in our patient deserves mention. There are few reports of cerebral infarcts occurring in patients with LSCS.

Calcified intracranial lesions may be produced by an inflammatory process in cerebral blood vessels. Interestingly enough, these lesions arise on the same side as the skin lesions, and there is still no reliable scientific explanation for this phenomenon.

The autoimmune hypothesis has the best evidence in its favour. It is based on reports of findings from cerebral biopsies showing inflammatory changes in the cerebral parenchyma, and sometimes in blood vessels and the meninges as well. In the case we present, we find evidence that the disease remains active; intracranial calcifications have continued growing slowly and gradually, despite use of correctly dosed immunosuppressants.

Transferencia de grasa autóloga en esclerodermia localizada y multicéntrica

Even considering the course of the disease and the presence of extracutaneous neurological signs, the patient has never met all the diagnostic criteria for systemic juvenile sclerosis. Please cite this article as: Esclerodermia lineal en golpe de sable y epilepsia. Presentation of a case in a ch Previous article Next article. September Pages Letter to the Editor.


Linear scleroderma en coup de sabre and epilepsy: Presentation of a case in a child. This item has received. A At 4 rsclerodermia of age and B at 7 years of age. Diagnosis and therapy of circumscribed scleroderma. J Dtsch Dermatol Ges, 6pp. Localized scleroderma associated with progressing ischemic stroke.

Rev Neurol, 41pp. Curr Opin Rheumatol, 18pp. Scleroderma en coup de sabre with intracranial involvement. Neurologia, 12pp. Arthritis Rheum, 57pp. Systemic sclerosis and localized scleroderma in childhood.

Rheum Dis Clin North Am, 34pp. Neurologic manifestations of localized scleroderma: Neurology, 71pp. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics,pp. Scand J Rheumatol, 35pp. Severe epilepsy preceding by four months the onset of scleroderma en coup de sabre. Clin Exp Rheumatol, 27pp. Circumscribed scleroderma — not just a harmless skin disease. Dtsch Med Wochenschr,pp. Feitosa de Oliveira, M. Rheumatology Oxford45pp. Linear scleroderma en coup de sabre: Pediatr Neonatol, 50pp.

Intracerebral involvement in scleroderma en coup de sabre: Ann Neurol, 37pp. Scleroderma en coup de sabre with central nervous system and ophthalmologic involvement: J Am Acad Dermatol, 49 escclerodermia, pp. Subscribe to our Newsletter. Print Send to a friend Export reference Mendeley Statistics.

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