Angiofibroma is a descriptive term for a group of lesions with different clinical presentations but with the same histopathology. These lesions include fibrous papule, facial angiofibroma, pearly penile papule, adenoma sebaceum, periungual fibroma, and Koenen’s tumor. This chapter will focus on facial angiofibroma. Generally, an angiofibroma presents as a 1 to 5 mm skin-colored to erythematous dome-shaped papule on the face. When it presents as multiple facial lesions, it can be associated with tuberous sclerosis or multiple endocrine neoplasia type 1 (MEN 1).
Age: majority in early to mid childhood
Precipitating factors: tuberous sclerosis, MEN 1
Patient with numerous facial angiofibromas. He is noted to have associated tuberous sclerosis
Firm skin-colored to erythematous papules (1–5 mm) on the nose, chin, and cheeks, which may be arranged bilaterally. Individuals with tuberous sclerosis can also have periungual fibromas, fibrous plaques, and ash-leaf macules.
Intradermal melanocytic nevi, appendageal tumors, basal cell carcinoma, acne vulgaris
A symmetric, well-circumscribed papule with a normal to slightly atrophic epidermis. The papillary and reticular dermis features a proliferation of varying degrees of normal blood vessels within a fibrotic stroma. The collagen fibers are arranged perpendicularly to the epidermis and concentrically around the vessels and hair follicles. Stellate-shaped multinucleated fibroblasts may be seen.
In the setting of multiple facial and/or periungual angiofibromas, tuberous sclerosis and MEN 1 must be investigated. This is best performed by referral to pediatric specialists.
Multiple facial angiofibromas typically present in childhood and may be associated with tuberous sclerosis (Fig. 40.2). Isolated lesions remain unchanged. Further angiofibromas may develop in adulthood.
(A) Fibrous plaques on the forehead in an adult patient with tuberous sclerosis. (B) Fibrous plaques on the scalp. (C) Ash leaf macule on the leg of the same patient
KEY CONSULTATIVE QUESTIONS
Onset and location of lesions
Family history of similar lesions
Family history of cancer
Associated central nervous system disorders
There is no medical indication to treat angiofibromas. Their cosmetic appearance, however, may be striking and understandably concerning to some individuals.
Multiple treatment modalities are available. Recurrence rate is high with the majority of the treatment options. Treatment options may be combined for the best ...