[Rosai-Dorfman Syndrome-a diverse clinical spectrum]

Rosai-Dorfman syndrome is a rare disease that belongs to the group of non-Langerhans cell histiocytosis. Affected patients can exhibit a wide variety of symptoms, which can be divided into nodal and extranodal manifestations. The syndrome primarily presents with fever, enlarged lymph nodes, and weight loss. Extranodal findings may include pancreatic cysts, hypergammaglobulinemia, and infiltration of testicular tissue. Skin involvement occurs in approximately one-third of cases. In addition to routine beside histopathology, immunohistochemistry with positivity for S100 and CD68 and negativity for CD 1a is particularly decisive for diagnosis. Topical and/or systemic steroids are used as first-line treatment for skin manifestations. In individual case studies, other systemic therapies have been used. Other treatment options include methotrexate (MTX), sirolimus, or rituximab. If KRAS/MAP2K1 mutation is detected, treatment with MEK inhibitors may be considered.