Chapter 40: Angiofibroma

INTRODUCTION

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).

EPIDEMIOLOGY

Incidence: common

Age: majority in early to mid childhood

Race: none

Sex: equal

Precipitating factors: tuberous sclerosis, MEN 1

PATHOGENESIS

Unknown.

PHYSICAL EXAMINATION

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.

DIFFERENTIAL DIAGNOSIS

Intradermal melanocytic nevi, appendageal tumors, basal cell carcinoma, acne vulgaris

DERMATOPATHOLOGY

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.

LABORATORY EXAMINATION

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.

COURSE

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.

KEY CONSULTATIVE QUESTIONS

• Onset and location of lesions

• Family history of similar lesions

• Family history of cancer

• Associated central nervous system disorders

MANAGEMENT

There is no medical indication to treat angiofibromas. Their cosmetic appearance, however, may be striking and understandably concerning to some individuals.

Treatment

Multiple treatment modalities are available. Recurrence rate is high with the majority of the treatment options. Treatment ...