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Indian Journal of Dermatology, Venereology and Leprology
Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL)
ISSN: 0378-6323
EISSN: 0378-6323
Vol. 74, No. 1, 2008, pp. 28-31
Bioline Code: dv08008
Full paper language: English
Document type: Research Article
Document available free of charge

Indian Journal of Dermatology, Venereology and Leprology, Vol. 74, No. 1, 2008, pp. 28-31

 en Forehead plaque: A cutaneous marker of CNS involvement in tuberous sclerosis
Rao, G. Raghu Rama; Rao , P.V. Krishna; Gopal, K.V.T; Kumar, Y. Hari Kishan & Ramachandra, B.V.

Abstract

Background: Tuberous sclerosis complex (TSC) is a neurocutaneous genodermatosis characterized by hamartoma formation in multiple organs. There are no definite cutaneous markers suggestive of central nervous system (CNS) involvement in TSC.
Aims: To study association of forehead plaque seen in tuberous sclerosis patients and CNS involvement in TSC.
Methods: This is a retrospective study of 15 cases of tuberous sclerosis in varying age groups - from 1.5 to 50 years. All the cases were thoroughly evaluated with detailed history; clinical examination; and relevant investigations like X-rays of chest, skull, hands and feet; ultrasound abdomen and computed tomography of brain.
Results: Out of the 15 cases, CNS involvement was seen in 8 cases. Seizures were present in 8 cases (53.33%) and mental retardation was seen in 6 cases (40%). Computerized tomography of brain revealed subependymal nodules (SENs) in eight cases (53.33%). In addition to SENs, subependymal giant cell astrocytomas and cortical tubers were seen in 2 cases each. Out of these 8 cases having CNS involvement, in 7 cases forehead plaque was observed. In 1 case, no forehead plaque was observed (X 2 = 1.07, P < 0.05).
Conclusion: This study shows that there is a statistically significant relationship between the presence of a forehead plaque and CNS involvement in TSC. Therefore, forehead plaque may be considered as a novel cutaneous marker to know the CNS involvement in TSC at an early stage.

Keywords
Central nervous system manifestations, fibrotic forehead plaque, Tuberous sclerosis complex

 
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Alternative site location: http://www.ijdvl.com

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