Mucocutaneous changes in tuberous sclerosis complex: A clinical profile of 27 Indian patients

• Main Authors: Ghosh Sudip (Author), Bandyopadhyay Debabrata (Author), Chatterjee Gobinda (Author), Ghosh Arghyaprasun (Author), Sarkar Sharmila (Author), Sarkar Somenath (Author)
• Format: Book
• Published: Wolters Kluwer Medknow Publications, 2009-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 0019-5154; 1998-3611
• Keywords: Tuberous sclerosis; mucocutaneous; adenoma sebaceum; shagreen patch; Dermatology; RL1-803
• Source: Indian Journal of Dermatology, Vol 54, Iss 3, Pp 255-257 (2009)

<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&#x0025;) patients. Adenoma sebaceum was the most common cutaneous feature (100&#x0025;), followed by hypomelanotic macules (92.6&#x0025;), connective tissue nevi (66.6&#x0025;), and Koenen&#x2032;s tumors (33.3&#x0025;). Oral mucosal fibromas were seen in six (22.22&#x0025;) 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.