Rosai–Dorfman disease complicated by autoimmune haemolytic anaemia: case report and review of a multisystem disease with cutaneous infiltrates

• Published in: British journal of dermatology (1951) Vol. 145; no. 2; pp. 323 - 326
• Main Authors: Grabczynska, S.A., Toh, C.T., Francis, N., Costello, C., Bunker, C.B.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Science Ltd 01.08.2001; Blackwell; Oxford University Press
• Subjects: Adult; Anemia, Hemolytic, Autoimmune - drug therapy; Anemia, Hemolytic, Autoimmune - etiology; Anti-Inflammatory Agents - therapeutic use; autoimmune haemolytic anaemia; Biological and medical sciences; Diabetes Mellitus, Type 2 - chemically induced; Female; Hematologic and hematopoietic diseases; Histiocytosis, Sinus - complications; Histiocytosis, Sinus - drug therapy; Humans; Medical sciences; orbits; Other diseases. Hematologic involvement in other diseases; Prednisolone - therapeutic use; Recurrence; Rosai–Dorfman disease; sinus histiocytosis with massive lymphadenopathy; sinuses; skin; Treatment Outcome; Case study; Human; Immunopathology; Hemolytic anemia; Sinus histiocytosis; Complication; Autoimmune disease; Hemopathy; Skin
• ISSN: 0007-0963; 1365-2133
• DOI: 10.1046/j.1365-2133.2001.04325.x

We report a patient with sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) who presented with widespread nodal and extranodal involvement affecting the skin, orbits and nasal sinuses, complicated by the development of autoimmune haemolytic anaemia. The aetiology and pathogenesis of this multisystem disorder are unknown but are thought to represent a reactive histiocytic process to an infective agent rather than a neoplastic or other primary condition. Prognosis is generally good but clinical or laboratory evidence of immune dysfunction tends to predict a poorer outcome. We describe the clinical course of the patient and review the literature on this disease.