Eccrine syringofibroadenoma: Multiple lesions representing a new cutaneous marker of the Schöpf syndrome, and solitary nonhereditary tumors - 11/09/11
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 bkeratoderma 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.
Conclusion: Multiple (palmoplantar) ESFAs are a new cutaneous marker of the Schöpf syndrome. (J Am Acad Dermatol 1997;36:569-76.)
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From the Department of Dermatology, Free University Hospital. |
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Reprint requests: Dr. Theo M. Starink, Department of Dermatology, Free University Hospital, P.O. Box 7057, 1007 MB Amsterdam, The Netherlands. |
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0190-9622/97/$5.00 + 0 16/1/78451 |
Vol 36 - N° 4
P. 569-576 - avril 1997 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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