Clouston syndrome associated with eccrine syringofibroadenoma

• Published in: Anais brasileiros de dermatología Vol. 89; no. 3; pp. 504 - 506
• Main Authors: de Andrade, Ana Carolina Dias Viana, Vieira, Denise Cardoso, Harris, Olga Maria Oiticica, Pithon, Matheus Melo
• Format: Journal Article
• Language: English
• Published: Sociedade Brasileira de Dermatologia 01-01-2014
• Subjects: Case Report
• ISSN: 0365-0596; 1806-4841
• DOI: 10.1590/abd1806-4841.20142996

Eccrine syringofibroadenoma is a rare benign neoplasia derived from acrosyryngium
cells of the eccrine sudoriferous glands. It affects the extremities of elderly
individuals as solitary tumors, or may also present as multiple lesions. There
are controversies about the pathogenesis and differentiation of the tumor.
Eccrine syringofibroadenoma has been associated with subjacent conditions, such
as for example, hypohidrotic ectodermic dysplasias. The authors describe a case
report of a patient with Clouston Syndrome, who presented papules and nodules in
extremities, clinically and histologically compatible with eccrine
syringofibroadenoma. There are only three cases described in the literature,
associated with Clouston Syndrome, and this is the fourth case.