Mucocutaneous changes in tuberous sclerosis complex: A clinical profile of 27 Indian patients
<b>Background:</b> Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disease resulting in a wide array of clinical manifestations, primarily affecting the skin and central nervous system. Mucocutaneous features play a very important role in the recognition of this...
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| Main Authors: | , , , , , |
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| Format: | Book |
| Published: |
Wolters Kluwer Medknow Publications,
2009-01-01T00:00:00Z.
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| Summary: | <b>Background:</b> Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disease resulting in a wide array of clinical manifestations, primarily affecting the skin and central nervous system. Mucocutaneous features play a very important role in the recognition of this syndrome. <b> Aims:</b> To review the prevalence and patterns of cutaneous manifestations in tuberous sclerosis, in a group of patients from eastern India. <b> Methods:</b> Observational clinical study on twenty-seven consecutive patients of tuberous sclerosis collected during a period of four years. <b> Results:</b> Most were between 10-20 years of age; the male to female ratio was 2:1. Family history was found in two-thirds. The classical triad of tuberous sclerosis was present in only nine (33.3%) patients. Adenoma sebaceum was the most common cutaneous feature (100%), followed by hypomelanotic macules (92.6%), connective tissue nevi (66.6%), and Koenen′s tumors (33.3%). Oral mucosal fibromas were seen in six (22.22%) patients. Fibromatous plaque over forehead and scalp was seen in three patients. Limitation of the study was small size of study sample. <b> Conclusion:</b> Prominent mucocutaneous changes are extremely common manifestation of TSC, which may provide crucial diagnostic clues for primary care physicians. |
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| Item Description: | 0019-5154 1998-3611 |