ArticleClinical medicine insights. Case reports2026
Primary Cutaneous Rosai-Dorfman Disease With Typical Immunophenotype But Lacking Emperipolesis: A Case Report.
Article in Clinical medicine insights. Case reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Background: Cutaneous Rosai-Dorfman disease (CRDD) is a rare extranodal histiocytosis that can mimic other dermatoses, particularly when hallmark emperipolesis is absent. Case Presentation: A 50-year-old man presented with 5 months of intensely pruritic lesions affecting the palms, soles, flexures, and limbs. Skin biopsy revealed a nodular infiltrate of foamy histiocytes extending into the subcutis. Immunohistochemistry showed S100⁺, CD68⁺, CD1a⁻, while emperipolesis was not identified. Systemic evaluation was unremarkable. He was treated with oral prednisone, which was discontinued due to steroid-induced hyperglycemia, and methotrexate 15 mg/week, achieving approximately 60% improvement of symptoms at 6 months. Conclusion: CRDD may present atypically, and the absence of emperipolesis does not exclude the diagnosis. Careful clinicopathologic correlation and immunohistochemical analysis are essential for accurate diagnosis and guiding individualized therapy.
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