ABSTRACT
Tuberous sclerosis complex (TSC) is a rare, genetically determined autosomal disorder that affects multiple organ systems. The skin is one of the target organs, and the most common skin lesions are hypopigmented macules that occur in more than 90% of patients with TSC. Other cutaneous clinical features include forehead plaques, shagreen patch, confetti-like macules, ungual fibromas, and facial angiofibromas. The vast majority of patients with TSC have one or more skin lesions, which usually develop early in life. Recognition of cutaneous lesions is important because they provide a visible clue to diagnosis. This chapter reviews the dermatological and other soft-tissue manifestations of TSC with special focus on the pigmentary abnormalities.
