Cutaneous Rosai-Dorfman disease

Abstract

BACKGROUND: Rosai-Dorfman disease is a sinus histiocytosis with massive lymphadenopathy. Histiocytes present emperipolesis, the characteristic phenomenon necessary for diagnosis. Etiology remains uncertain, it has been linked to immune dysfunction and some infectious agents. It can affect any organ of the economy, becoming a deadly disease when it infiltrates vital organs. Exclusive skin disease is extremely rare, occurring only in 3% of cases. Dermatological manifestations are polymorphic, making diagnosis difficult and sometimes mimicking other more common dermatological conditions. Treatment can be expectant or with different drugs, surgery, radiotherapy or chemotherapy. CLINICAL CASE: A 43-year-old female patient with lesions in the left pinna and scapular area; patient had a remarkable response to systemic treatment with chemotherapy. CONCLUSIONS: The coexistence of histiocytes positive for the markers S-100 + and negative for CD1a-, with emperipolesis, warrants the diagnosis of the disease.