Nevus Sebaceus and Follicular Hamartomas

 Usually presents on scalp in infancy, often at birth


image Presents as yellowish flat to warty plaque; enlarges during puberty


• Basaloid follicular hamartoma
image Usually presents in adults




Microscopic




• Nevus sebaceus
image Abnormalities of epidermis, follicles, apocrine and sebaceous glands

– Proliferating sebaceous glands high in dermis; may communicate with epidermal surface

image Follicles are disorganized with primitive germs

image Associated tumors arising within nevus sebaceus

– Trichoblastoma (most common), syringocystadenoma papilliferum, basal cell carcinoma (BCC) (rare)

• Basaloid follicular hamartoma
image Multifocal islands of basaloid epithelium

image No retraction artifact; no to rare mitotic activity


Top Differential Diagnoses




• DDx of nevus sebaceus
image Epidermal nevus: Lacks abnormalities of follicles and sweat glands

image Sebaceous adenoma: Lacks epidermal acanthosis and follicular structures

image Sebaceous hyperplasia: Mature follicles without epidermal changes

• DDx of basaloid follicular hamartoma
image BCC: More cytologic atypia, retraction spaces, inflammatory stroma, mitotic activity

image
Nevus Sebaceus
Nevus sebaceus has an acanthotic epidermis, often with a papillomatous surface image. The follicles have a disorganized appearance image, and there are often associated large sebaceous glands image.


image
Basaloid Follicular Hamartoma
Basaloid follicular hamartoma is characterized by multifocal islands of basaloid epithelium image resembling primitive follicles that are surrounded by a fibroblastic stroma.

image
Nevus Sebaceus at Low Magnification
This nevus sebaceus demonstrates a papillomatous acanthotic epidermis image overlying disorganized follicles with prominent sebaceous glands image.

image
Immature Follicles in Nevus Sebaceus
In this nevus sebaceus from a young child, there are disorganized, immature follicles image; sebaceous glands are not prominent in this view but are often hypoplastic before puberty.


TERMINOLOGY


Synonyms




• Nevus sebaceus: Organoid nevus, Jadassohn nevus


Definitions




• Nevus sebaceus
image Hamartoma with abnormalities of epidermis, follicles, sebaceous glands, and apocrine glands

• Basaloid follicular hamartoma
image Hamartoma resembling primitive follicles


ETIOLOGY/PATHOGENESIS


Developmental Anomaly




• Nevus sebaceus
image Deletion of PTCH1 (PTCH) gene may play role

• Basaloid follicular hamartoma
image Possible role for Sonic Hedgehog signaling pathway


CLINICAL ISSUES


Presentation




• Nevus sebaceus
image Usually presents in infancy, often at birth; often enlarges during puberty

image Usually involves scalp, less commonly on face, as yellowish flat to warty plaque

• Follicular hamartomas
image Solitary
– Elderly patients with flesh-colored papule on face

– May clinically mimic basal cell carcinoma (BCC)

image Localized
– Presents in 3rd-4th decade on head as erythematous to hyperpigmented plaque

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Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Nevus Sebaceus and Follicular Hamartomas

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