Syringocystadenoma papilliferum
SCAP; syringocystadenoma papilliferum
Syringocystadenoma papilliferum is a benign adnexal tumour of apocrine and/or eccrine differentiation, classically arising in a pre-existing naevus sebaceus of Jadassohn on the scalp or face in childhood or adolescence. It presents as a pink, weeping, papillomatous papule or plaque that may discharge serous fluid. Histology shows characteristic papillary projections lined by two layers of epithelium with a dense plasma-cell infiltrate. BRAF-mutation overlap is recognised. Complete excision is curative; rare malignant transformation (syringocystadenocarcinoma papilliferum) has been reported.
Clinical features
- Solitary pink or red papule, plaque or nodule with verrucous, papillomatous surface, sometimes with serous discharge or crusting.
- Distribution — scalp (commonest), face, neck; chest, axilla.
- Often arises within a pre-existing naevus sebaceus on the scalp at puberty.
- Occasionally congenital or appearing in early adulthood.
- May ulcerate or bleed with trauma.
Histology
- Cystic invaginations from the surface epidermis into the dermis, lined by papillary projections of two-layered epithelium — luminal columnar cells with decapitation secretion and outer cuboidal cells.
- Dense plasma-cell-rich infiltrate within the papillary stroma is highly characteristic.
- BRAF V600E and HRAS mutations have been described.
- Differential — hidradenoma papilliferum, tubular apocrine adenoma, syringocystadenocarcinoma papilliferum (malignant counterpart with atypia/infiltration).
Context — naevus sebaceus association
- Approximately 5–15% of naevus sebaceus develop secondary neoplasms; syringocystadenoma papilliferum and trichoblastoma are the commonest benign secondary tumours.
- BCC and other malignancies are rare but recognised.
- This is the rationale for prophylactic excision of naevus sebaceus around puberty, although modern practice often defers excision to symptomatic change.
Management
- Complete surgical excision with histology.
- If arising within naevus sebaceus — excise the entire naevus with a small margin.
- Reassurance after complete excision for confirmed benign disease.
- Suspicious features (rapid growth, ulceration, infiltration) — exclude syringocystadenocarcinoma papilliferum; wide local excision with MDT discussion.
References
- Helwig EB, Hackney VC. Syringocystadenoma papilliferum. Arch Dermatol; 1955.
- Idriss MH, Elston DM. Secondary neoplasms within naevus sebaceus — a review. J Am Acad Dermatol; 2014.
- WHO Classification of Tumours Editorial Board. Skin Tumours, WHO Classification of Tumours, 5th ed., vol. 12. Lyon: IARC; 2025.
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