RESUMO
Circumscribed storiform collagenoma is a rare benign tumor. It appears as an isolated skin lesion or as part of the clinical spectrum of Cowden syndrome. The pathogenesis is still controversial. Although its clinical expression is heterogeneous, it has a characteristic histological pattern. We describe a case of a solitary circumscribed storiform collagenoma not associated with Cowden syndrome.
Assuntos
Fibroma/patologia , Neoplasias Cutâneas/patologia , Doenças do Colágeno/patologia , Feminino , Dedos/patologia , Humanos , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Langerhans cell histiocytosis (LCH) is a neoplasm of the monocyte-macrophage lineage, characterized by clonal proliferation and dissemination of cells that express CD1a+ and CD207. It is a disorder that predominates in childhood. Although the skin is the second most frequently affected organ (30-60%), isolated cutaneous involvement is rare; its frequency does not exceed 4 to 12 percent of cases. Single system-LCH usually has a good prognosis. We describe a case of LCH with isolated cutaneous involvement that presented in an adult patient and was refractory to polychemotherapy.
