T M Starink1. 1. Department of Dermatology, Free University Hospital, Amsterdam, The Netherlands.
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. CONCLUSION: Multiple (palmoplantar) ESFAs are a new cutaneous marker of the Schöpf syndrome.
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. CONCLUSION: Multiple (palmoplantar) ESFAs are a new cutaneous marker of the Schöpf syndrome.
Authors: Christian P Vink; Charlotte W Ockeloen; Sietske ten Kate; David A Koolen; Johannes Kristian Ploos van Amstel; Anne-Marie Kuijpers-Jagtman; Celeste C van Heumen; Tjitske Kleefstra; Carine E L Carels Journal: Eur J Hum Genet Date: 2014-01-08 Impact factor: 4.246
Authors: Iga Płachta; Marcin Kleibert; Anna M Czarnecka; Mateusz Spałek; Anna Szumera-Ciećkiewicz; Piotr Rutkowski Journal: Int J Mol Sci Date: 2021-05-11 Impact factor: 5.923