Facial Angiofibromas Associated with Tuberous Sclerosis

Angiofibromas are small reddish spots or bumps that consist of fibrous tissue and blood vessels. They are most commonly found around the nose, cheeks, and chin, often combining to form a distinctive butterfly-shaped pattern” (Tuberous Sclerosis, 2009). Angiofibromas typically appear in people with TSC between the ages of two and five, but may appear earlier or later in life.

“Skin lesions, which are found in 70-80 percent of cases are important features for early diagnosis and constitute four major features and one minor feature in the diagnostic criteria” (Wee & Fangman, 2007).

“Early on, angiofibromas tend to be relatively bright red and level with the skin surface, but over time, they become thicker, more fibrous, and more numerous. Although angiofibromas are unique to TSC and considered a major feature in the diagnostic criteria, they are sometimes mistaken for other skin disorders, including acne. Also, because angiofibromas seldom appear in children younger than two years of age, they are of little use in diagnosing TSC in infants and very young children.

The most commonly treated TSC-related skin lesions are facial angiofibromas. Treatment becomes important especially for adolescents and young adults when the lesions become more pronounced and more psychologically distressing as a result. In young men, blood-rich angiofibromas can also bleed significantly when they are nicked during shaving” (Corporation, 2006).

“Angiofibromas can present a cosmetic deformity and difficulties with hygiene and nasal breathing. Various approaches to treatment have been described over the last century, but no long-term results have been presented. Because of the full-thickness skin involvement, complete removal is not practical, and treatment should be directed toward acceptable long-term results with minimal surgical morbidity.

Shaving and dermabrasion of the involved area produce very satisfactory results, but long-term follow-up of approximately 10 years reveals that there is a variable amount of recurrence and that subsequent treatment will be required” (U.S. National Library of Medicine, n.d.).

source Dermatology Atlas ©(2001-2011). Contributor Yahia Albail, DO