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