title: Eccrine syringofibroadenoma: multiple lesions representing a new cutaneous marker of the Schöpf syndrome, and solitary nonhereditary tumors.
abstract: 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.
authors.affiliation: Department of Dermatology, Free University Hospital, Amsterdam, The Netherlands.
authors.forename: T M
authors.initials: TM
authors.lastname: Starink
mesh_terms: 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*