Year: 2026
Type: article
Study design: Case Report
Abstract: Rosai–Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a non-Langerhans-type histiocytic disorder characterized by histiocyte proliferation with emperipolesis. Rosai-Dorfman disease is a rarely diagnosed disease with variable clinical manifestations on the skin, in the lymphatic system, internal organs, requiring differential diagnosis with such nosologies as sarcoidosis, Langerhans cell histiocytosis, vasculitis, lymphoproliferative diseases (Hodgkin's lymphoma, non-Hodgkin's lymphomas). According to modern concepts, the disease can be associated with immunodeficiency states, including LRBA deficiency, and also demonstrate a variety of extranodal manifestations - from isolated axillary lymphadenopathy to destructive lesions of the bone structures of the nose with the formation of a recession of the nasal dorsum. Difficulties in differential diagnosis are associated with the similarity of certain histological and clinical features and molecular changes. Immunohistochemical testing with detection of the S-100 and CD163 markers, which are key diagnostic criteria for differentiating this pathology from other histiocytoses, is mandatory for confirming the diagnosis. This article describes Rosai-Dorfman disease in a 42-year-old man. The diagnosis was difficult for a dermatologist during an outpatient clinic visit. The initial clinical misdiagnosis (without histological or immunohistochemical examination) of benign lymphocytic keratosis led to an inappropriate management strategy. This clinical case demonstrates the challenges of differential diagnosis of Rosai-Dorfman disease in a dermatologist's routine practice. In the Russian Federation, systematic epidemiological data on the incidence of Rosai-Dorfman disease have been absent from the available literature in recent years. Existing publications consist of isolated clinical observations, due to the disorder's orphan status and the lack of a centralized federal registry of patients with this disease. International literature estimates the incidence to be less than 1 case per 1,000,000 population per year. Reporting each clinical observation is essential for accumulating data and optimizing the diagnosis and treatment of this rare disease.
DOI: 10.17816/dv717529
Language: English
Subfield: Physiology (medical)
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W7222686171