Clinical challenges in the diagnosis and management of disseminated Rosai-Dorfman Disease presenting with chronic severe anemia: a case report

Abstract

Rosai–Dorfman Disease (RDD) is an uncommon non-Langerhans cell histiocytic disorder, with varying clinical presentations and often complicate diagnosis, particularly in extranodal cases resembling other medical conditions. Moreover, standard treatment guidelines have not been well-established due to its rarity. We report the case of a 59-year-old woman, initially presenting with severe anemia and subsequent bone pain, hypercalcemia, renal impairment, small lymphadenopathy, and ulcerated nodular skin lesions. Histopathologic examination of a skin biopsy revealed histiocytic cell infiltration that was stained positively for S100 and CD68 but negatively for CD1a and Langerin, supporting the diagnosis of RDD. Following corticosteroid therapy combined with mercaptopurine, the cutaneous lesions showed a favorable response, and all laboratory parameters improved progressively during follow-up.