Sinus histiocytosis with massive lymphadenopathy (Rosai and Dorfman) and significant skin involvement

Abstract

Clinicopathological features of this female patient described here, showing unusual and extremely marked sinus histiocytosis of lymph nodes with considerable systemic lymphadenopathy, accompanying fever as well as acceleration of erythrocyte sedimentation rate fairly well coincided with the symptoms of "Sinus Histiocytosis With Massive Lymphadenopathy" (Rosai and Dorfman). The onset of the present case was in 1966 when the patient was 10 years of age, and she has been followed-up for over 10 years. While persisting to display active clinical manifestations, immunological abnormality with thymus involution was demonstrated--deterioration of cell-mediated immunity, but with non-remarkable humoral immunological data, except for hypergammaglobulinemia with elevation of IgG. In 1973 lymphadenopathy as well as skin eruption extended over the extremities and abnormalities of hematological and serological nature began to improve and at the present date the patient is uneventful. This is the first recorded case of "Sinus Histiocytosis With Massive Lymphadenopathy" (Rosai and Dorfman) in Japan. Considerable skin involvement in the upper and lower extremities was a significant feature and unique in this case.