Usually presents on scalp in infancy, often at birth
Presents as yellowish flat to warty plaque; enlarges during puberty
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Basaloid follicular hamartoma
Usually presents in adults
Microscopic
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Nevus sebaceus
Abnormalities of epidermis, follicles, apocrine and sebaceous glands
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Proliferating sebaceous glands high in dermis; may communicate with epidermal surface
Follicles are disorganized with primitive germs
Associated tumors arising within nevus sebaceus
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Trichoblastoma (most common), syringocystadenoma papilliferum, basal cell carcinoma (BCC) (rare)
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Basaloid follicular hamartoma
Multifocal islands of basaloid epithelium
No retraction artifact; no to rare mitotic activity
Top Differential Diagnoses
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DDx of nevus sebaceus
Epidermal nevus: Lacks abnormalities of follicles and sweat glands
Sebaceous adenoma: Lacks epidermal acanthosis and follicular structures
Sebaceous hyperplasia: Mature follicles without epidermal changes
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DDx of basaloid follicular hamartoma
BCC: More cytologic atypia, retraction spaces, inflammatory stroma, mitotic activity
TERMINOLOGY
Synonyms
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Nevus sebaceus: Organoid nevus, Jadassohn nevus
Definitions
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Nevus sebaceus
Hamartoma with abnormalities of epidermis, follicles, sebaceous glands, and apocrine glands
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Basaloid follicular hamartoma
Hamartoma resembling primitive follicles
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
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Nevus sebaceus
Deletion of
PTCH1 (PTCH) gene may play role
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Basaloid follicular hamartoma
Possible role for Sonic Hedgehog signaling pathway
CLINICAL ISSUES
Presentation
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Nevus sebaceus
Usually presents in infancy, often at birth; often enlarges during puberty
Usually involves scalp, less commonly on face, as yellowish flat to warty plaque
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Follicular hamartomas
Solitary
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Elderly patients with flesh-colored papule on face
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May clinically mimic basal cell carcinoma (BCC)