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| LETTER TO EDITOR |
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| Year : 2014 | Volume
: 5
| Issue : 1 | Page : 91 |
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Tuberous sclerosis complex
Namitha Chathra, M Ramesh Bhat
Department of Dermatology, Father Muller Medical College, Kankanady, Mangalore, Karnataka, India
| Date of Web Publication | 15-Mar-2014 |
Correspondence Address:
Namitha Chathra
Department of Dermatology, Father Muller Medical College, Kankanady, Mangalore - 575 002, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0975-9727.128969
How to cite this article:
Chathra N, Bhat M R. Tuberous sclerosis complex. Muller J Med Sci Res 2014;5:91 |
Dear Editor,
A 28-year-old male with insignificant family history presented with asymptomatic reddish raised lesions on the face and neck, which were first noticed by him when he was 8 years old. There is no history suggestive of systemic involvement or progression of the lesions. On examination, several reddish-brown papules of varying sizes were noted [Figure 1]a which were confirmed as angiofibromas with the aid of histology. He was also found to have multiple randomly distributed dental enamel pits [Figure 2]a. With a diagnosis of tuberous sclerosis in mind, he was evaluated further. Fundus examination revealed left astrocytoma [Figure 2]b, computed tomography scan of the brain showed a few calcified subependymal nodules [Figure 2]c, ultrasonography abdomen was suggestive of adrenal lipoma [Figure 2]d and echocardiogram did not detect any abnormality. A definite diagnosis of tuberous sclerosis was made based on the presence of two major features. [1] The skin lesions were ablated using carbon dioxide laser [Figure 1]b but patient refused further evaluation and management of systemic lesions. | Figure 1: (a) Multiple angiofibromas on the face. (b) Post ablation with CO2 laser
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 | Figure 2: (a) Multiple randomly distributed dental enamel pits. (b) Fundus examination-left astrocytoma. (c) Computed tomography scan of the braincalcified subependymal nodules. (d) Ultrasonography abdomen-adrenal lipoma
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Tuberous sclerosis complex is an autosomal dominant multisystem hamartomatosis involving mainly the skin, eye, central nervous system, heart, lungs, kidneys and bones. The incidence is as high as 1 in 6000 live births and the affected individuals exhibit decreased overall survival in comparison with the general population, although some may have a normal life span with few medical complications. [2]
| References | |  |
| 1. | Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: Revised clinical diagnostic criteria. J Child Neurol 1998;13:624-8. 
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| 2. | Osborne JP, Fryer A, Webb D. Epidemiology of tuberous sclerosis. Ann N Y Acad Sci 1991;615:125-7.
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[Figure 1], [Figure 2]
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