Muller Journal of Medical Sciences and Research

LETTER TO EDITOR
Year
: 2014  |  Volume : 5  |  Issue : 1  |  Page : 91-

Tuberous sclerosis complex


Namitha Chathra, M Ramesh Bhat 
 Department of Dermatology, Father Muller Medical College, Kankanady, Mangalore, Karnataka, India

Correspondence Address:
Namitha Chathra
Department of Dermatology, Father Muller Medical College, Kankanady, Mangalore - 575 002, Karnataka
India




How to cite this article:
Chathra N, Bhat M R. Tuberous sclerosis complex.Muller J Med Sci Res 2014;5:91-91


How to cite this URL:
Chathra N, Bhat M R. Tuberous sclerosis complex. Muller J Med Sci Res [serial online] 2014 [cited 2021 Dec 2 ];5:91-91
Available from: https://www.mjmsr.net/text.asp?2014/5/1/91/128969


Full Text

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}{Figure 2}

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

1Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: Revised clinical diagnostic criteria. J Child Neurol 1998;13:624-8.
2Osborne JP, Fryer A, Webb D. Epidemiology of tuberous sclerosis. Ann N Y Acad Sci 1991;615:125-7.