Rosai-Dorfman Disease associated with Myasthenia Gravis

Abstract

We present the case of a 29-year-old patient with a prior diagnosis of myasthenia gravis at age 20, who years later developed skin lesions compatible with Rosai-Dorfman disease on histopathological testing. She initially had a stable lesion on her right auricle, and then progressed with new lesions on her right buttock, thigh and knee. With the onset of multifocal, recurrent lesions, oral corticosteroid therapy cycles were started, achieving a transient reduction in the size and color of the lesions, but with recurrence when the treatment was stopped. Currently, the patient is stable, having received multiple corticosteroid cycles over the last few years. The association of these two conditions is extremely rare and points to a possible immunopathological interrelationship. This case illustrates the difficulties in effective long-term management of the skin lesions associated with Rosai-Dorfman disease, which in this particular case required multiple corticosteroid cycles with a transient benefit. Studies of more sustainable treatments and/or with a better response profile are needed. Communicating these rare cases encourages the exchange of knowledge and experiences among specialists