ECCRINE SYRINGOFIBROADENOMA - MULTIPLE LESIONS REPRESENTING A NEW CUTANEOUS MARKER OF THE SCHOPF SYNDROME, AND SOLITARY NONHEREDITARY TUMORS

Background: Eccrine syringofibroadenoma (ESFA) is a rare eccrine tumor that has characteristic histopathologic features and variable clinica l findings. Objective: Our purpose was to define the clinical and hist opathologic features of ESFA, to assess a possible syndromic associati on and heredity, and to propose a clinicopathology classification. Met hods: Three solitary and five multiple cases of ESFA were analyzed and the findings compared with those of previously published cases. Resul ts: Clinically, solitary ESFA was typically a nonhereditary verrucous growth. Multiple examples presented as palmoplantar keratoderma and ke ratotic papules in a mosaic pattern. In the multiple form, four patien ts had the Schopf syndrome (hydrocystomas of the eyelids, hypotrichosi s, hypodontia, and nail abnormalities); one had an incomplete form of the syndrome. Histologically, ESFA showed a reticulate proliferation o f thin strands of acrosyringeal cells with focal lumen formation, and a fibrovascular stroma. Malignant ESFA was present in one multiple cas e. Conclusion: Multiple (palmoplantar) ESFAs are a new cutaneous marke r of the Schopf syndrome.