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An Bras Dermatol. 2014 May-Jun;89(3):504-6.

Clouston syndrome associated with eccrine syringofibroadenoma.

Author information

Universidade Estadual do Sudoeste da Bahia, Jequié, BA, Brazil.
Centro Municipal de Atenção Especializada, Vitória da Conquista, BA, Brazil.
Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.

Erratum in

  • An Bras Dermatol. 2014 Jul-Aug;89(4):692.


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.

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