Eccrine syringofibroadenoma: multiple lesions representing a new cutaneous marker of the Schöpf syndrome, and solitary nonhereditary tumors.
BACKGROUND
Eccrine syringofibroadenoma (ESFA) is a rare eccrine tumor that has characteristic histopathologic features and variable clinical findings.


OBJECTIVE
Our purpose was to define the clinical and histopathologic features of ESFA, to assess a possible syndromic association and heredity, and to propose a clinicopathologic classification.


METHODS
Three solitary and five multiple cases of ESFA were analyzed and the findings compared with those of previously published cases.


RESULTS
Clinically, solitary ESFA was typically a nonhereditary verrucous growth. Multiple examples presented as palmoplantar keratoderma and keratotic papules in a mosaic pattern. In the multiple form, four patients had the Schöpf syndrome (hydrocystomas of the eyelids, hypotrichosis, hypodontia, and nail abnormalities); one had an incomplete form of the syndrome. Histologically, ESFA showed a reticulate proliferation of thin strands of acrosyringeal cells with focal lumen formation, and a fibrovascular stroma. Malignant ESFA was present in one multiple case.


CONCLUSIONS
Multiple (palmoplantar) ESFAs are a new cutaneous marker of the Schöpf syndrome.
Starink
T M
TM
Department of Dermatology, Free University Hospital, Amsterdam, The Netherlands.
D006607:Adenoma, Sweat Gland / Q000473:pathology*; D000368:Aged; D001706:Biopsy; D005142:Eyelid Neoplasms / Q000473:pathology; D005260:Female; D005528:Foot; D006225:Hand; D006801:Humans; D008875:Middle Aged; D013544:Sweat Gland Neoplasms / Q000473:pathology*