Syringoma is a benign adnexal
tumour.
These lesions are common in patients with Down syndrome.
Clear cell syringoma are commonly associated with diabetes mellitus.
Clinical presentation:
i) Multiple papules on
the lower eyelids and cheeks of adolescent females ii) Solitary & giant lesions, plaque form or milia-like
lesions limited to the vulva, penis, buttock , neck , scalp,
axilla, abdomen or extremities.
Microscopic features:
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Dermal tumours are composed of small ducts lined by
two layers of cuboidal epithelium ; ducts have a comma-like tail ; solid nests and strands of basaloid cells
may be present in the dermis ; some ducts contain eosinophilic material
; backround
stroma is usually fibrotic ; the lesion is well demarcated from the
surrounding dermis ; numerous mast cells are present in the stroma in
some cases.
Clear cell variant-
ducts are lined by epithelial cells with pale or clear cytoplasm.
Immunohistochemistry:
Tumour cells express cytokeratin10 in the
intermediate cells, cytokeratin 6 and 19 and CEA in the luminal cells
and EMA in the peripheral cells of the duct. ( D/D- CEA is negative in
desmoplastic trichoepithelioma).
Progesterone receptors are expressed in most syringomas.
Differential diagnosis:
Microcystic
adnexal carcinoma
(no perineural invasion or deep extension in syringoma). It is
impossible to make the distinction in a shave biopsy.
Chondroid syringoma.
DermAtlas
(clinical):
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Image
Link( Dr Weems):
CLICK HERE
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