Angiofibroma and Related Lesions
Steven D. Billings, MD
Key Facts
Clinical Issues
Fibrous papule
Adenoma sebaceum: Angiofibromas associated with tuberous sclerosis
Solitary dome-shaped flesh-colored papules on nose or central face
May clinically mimic basal cell carcinoma
Microscopic Pathology
Involves superficial dermis
Scattered bland, spindled to stellate, and multinucleated fibroblasts
Dense collagen
Ectatic thin-walled blood vessels
Top Differential Diagnoses
Dermatofibroma
Scar
Xanthogranuloma
Epithelioid fibrous histiocytoma
 Fibrous papule is characterized by a dome-shaped lesion with ectatic thin-walled blood vessels  and dense collagenous stroma. |
 This high-power image of a fibrous papule demonstrates the relatively hypocellular proliferation of bland spindled to stellate fibroblasts, small ectatic vessels, and dense collagenous stroma. |
TERMINOLOGY
Synonyms
Fibrous papule (FP), adenoma sebaceum, acral angiofibroma, pearly penile papule
Definitions
Angiofibroma encompasses a group of benign mesenchymal tumors characterized by spindled to stellate fibroblasts, dense collagenous stroma, and ectatic blood vessels
May be related to a proliferation of papillary dermal elements
CLINICAL ISSUES
Presentation
Fibrous papule
Middle-aged patients
Solitary dome-shaped flesh-colored papules on nose or central face
May clinically mimic basal cell carcinoma
Adenoma sebaceum
Angiofibromas associated with tuberous sclerosis
Tuberous sclerosis: Autosomal dominant inheritance, but most cases sporadic
Mutation in TSC1 or TSC2
Term is a misnomer, as there is no proliferation of sebaceous glands
Grouped papules or nodules with butterfly distribution on central face
Pearly penile papules
Grouped or linear white papules on coronal margin &/or sulcus of penis
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