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Linear Scleroderma en Coup de Sabre and Brain Calcification: Is There a Pathogenic Relationship?
To the Editor: We read with interest the report by Flores-Alvarado, et al1 describing a case of scleroderma en coup de sabre (SCS) and brain calcification. The authors note this condition is uncommon and there is no consensus on the association between the 2 conditions. We describe a child with similar clinical findings. A 6-year-old girl was admitted to our clinic due to skin discoloration and depression on her right forehead and chin. She had been followed by the pediatric neurology department because of her epileptic seizures, and had been receiving carbamazepine and sodium valproate treatment for 2 years. Her family noted echymotic color change and a depression becoming progressively more prominent affecting her forehead and chin within one year. Her history revealed she had had seizures (secondary generalized tonic clonic, with right eye and head deviation) with onset 2 years before, which were controlled by antiepileptic therapy. She was the second child of non-consanguineous healthy parents and she had a healthy brother. No previous similar case was described in her family. On physical examination her height was 112 cm (90-97th percentiles), weight was 26 kg (97th percentile), head circumference was 54.5 cm (2-50th percentile), and blood pressure was 90/65 mm Hg. There was skin thinning and darkening of the skin color on the right forehead starting from the brow through the scalp (2 � 3 cm area) and on the right chin under the lip commissure (1 � 3 cm). There was bone depression and atrophy beneath these affected skin regions (Figure 1). There was no glossal or buccal atrophy. Other systemic findings were ordinary.  Figure 1. Scleroderma en coup
de sabre lesion involving the right forehead.
Complete blood count, erythrocyte sedimentation rate, and biochemical findings of the serum were within normal ranges. Rheumatoid factor and antinuclear antibodies were negative. Bone scintigraphy was normal. She had had normal findings on her cranial magnetic resonance imaging examination 2 years before, but cranial computerized tomographic imaging demonstrated multiple intraparenchymal calcifications, predominantly in the left hemisphere localization, and there was thinning on her right mandible and right frontal calvarium (Figure 2). There were no sclerotic or lytic lesions of the bone structure. Bioelectrical disturbances were observed on the posterior region of the right hemisphere.  Figure 2. Axial computer
tomography scan shows intracerebral calcifications.
Prednisolone (10 mg/kg/day) and methotrexate (10 mg/m2/week) treatment was prescribed on a diagnosis of SCS with right facial progressive atrophy and linear scleroderma. After 6 months of treatment minimal fading of the skin lesions was observed. Linear scleroderma is the most common subtype of localized scleroderma in children and adolescents, and is characterized by one or more linear streaks that typically involve an upper or lower extremity and may be associated with morphea plaques2. When a linear lesion involves the face or scalp, it is referred to as SCS2-5. Differential diagnosis of SCS should include the Parry-Romberg syndrome as stated by authors (1,4). In most cases with SCS and Parry-Romberg syndrome, disorders such as seizures, uveitis, dental abnormalities and ocular muscle dysfunction, loss of eyebrows or eyelashes, and atrophy of the iris have been described, in addition to the abnormalities noted above4,6-8. In adult cases having localized scleroderma, intracranial calcification together with seizures are reported rarely1. In 37 reported cases with systemic sclerosis, 16 (43%) had seizures, and 12 patients with seizures had intracerebral calcifications8. SCS having obscure etiology and pathogenesis may be observed in cases of childhood epilepsy, and seizures may occur before facial findings3-5. There is no intracranial pathology accompanying seizures4. There are few reports of SCS cases in childhood. In the largest series consisting of 13 cases there was no intracerebral calcification (IC)4. In another study of SCS, despite the observation of seizures, no IC was reported3. Liu, et al5 described IC in 2 SCS cases in a series of 23 cases of localized scleroderma. Thus, calcification is very rare in childhood SCS3-5. The above data suggest that the coupling of SCS and IC may represent a new syndrome. In any case with SCS and seizure, cranial imaging studies should be performed. �ZG�R KASAP�OPUR, MD, Atak�y 4, Kisim 0-117/4 34750 Istanbul; HILDA �ER�I �ZKAN, MD; BEYHAN T�YS�Z, MD, Department of Pediatrics, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey. E-mail: ozgurcopur@e-kolay.net REFERENCES 1. Flores-Alvarado DE, Esquivel-Valerio JA, Garza-Elizondo M, Espinoza LR. Linear scleroderma en coup de sabre and brain calcification: Is there a pathogenic relationship? J Rheumatol 2003;30:193-5. 2. Nelson AM. Linear scleroderma. In: Cassidy JT, Petty RE, editors. Textbook of pediatric rheumatology. Philadelphia: WB Saunders; 2001:536-43. 3. David J, Wilson J, Woo P. Scleroderma 'en coup de sabre'. Ann Rheum Dis 1991;50:260-2. 4. Orozco-Covarrubias L, Guzman-Meza A, Ridaura-Sanz C, Carrasco Daza D, Sosa-de-Martinez C, Ruiz-Maldonado R. Scleroderma 'en coup de sabre' and progressive facial hemiatrophy. Is it possible to differentiate them? J Eur Acad Dermatol Venereol 2002;16:361-6. 5. Liu P, Uziel Y, Chuang S, Silverman E, Krafchik B, Laxer R. Localized scleroderma: imaging features. Pediatr Radiol 1994;24:207-9. 6. Dupont S, Catala M, Hasboun D, Semah F, Baulac M. Progressive facial hemiatrophy and epilepsy: A common underlying dysgenetic mechanism. Neurology 1997;48:1013-7. 7. Derex L, Isnard H, Revol M. Progressive facial hemiatrophy with multiple benign tumors and hamartomas. Neuropediatrics 1995;26:306-9. 8. Heron E, Hernigou A, Chatellier G, Fornes P, Emmerich J, Fiessinger JN. Intracerebral calcifications in systemic sclerosis. Stroke 1999;30:2183-5. Dr. Flores-Alvarado, et al, reply To the Editor: We appreciate the thoughtful comments of Kasapcopur, et al on our recent case report. From their review of the literature, they propose that the association of linear scleroderma en coup de sabre (SCS) and intracranial calcification represents a new syndrome or pathogenic coexistence, and further recommend cranial imaging studies in SCS and seizure1. At present, however, there is limited evidence to support such a conclusion. SCS is an unusual disorder, and in contrast to adult scleroderma there are no available prospective imaging studies of SCS patients to detect intracranial calcification. Regarding the study of Orozco-Covarrubias, et al2 involving a fairly large number of SCS patients (n = 13), and also Parry-Romberg patients (n = 9), it did not specifically address this issue, and it was not clear whether imaging studies were performed in all patients. However, we agree with the second part of their conclusion, although we would expand it to include cranial imaging studies in all SCS patients exhibiting any type of clinical neurologic involvement. DIANA ELSA FLORES-ALVARADO, MD; JORGE A. ESQUIVEL-VALERIO, MD; MARIO GARZA-ELIZONDO, MD, Section of Rheumatology, Department of Medicine, Universidad Autonoma de Nuevo Leon, Monterrey, Mexico; LUIS R. ESPINOZA, MD, Professor and Chief, Section of Rheumatology, Louisiana State University Health Sciences Center, New Orleans, Louisiana 70112, USA. REFERENCES 1. Heron E, Hernigou A, Chatellier G, Fornes P, Emmerich J, Fiessinger JN. Intracerebral calcifications in systemic sclerosis. Stroke 1999;30:2183-5. 2. Orozco-Covarrubias L, Guzman-Meza A, Ridaura-Sanz C, Carrasco Daza D, Sosa-de-Martinez C, Ruiz-Maldonado R: Scleroderma 'en coup de sabre' and progressive facial hemiatrophy. Is it possible to differentiate them? J. Eur Acad Dermatol Venereol 2002;16:361-6. |