Rosai-Dorfman disease complicated by autoimmune haemolytic anaemia: case report and review of a multisystem disease with cutaneous infiltrates.
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.
Grabczynska
S A
SA
Department of Dermatology, Chelsea Hospital, Fulham Road, London SW10 9NH, UK.
Toh
C T
CT
Francis
N
N
Costello
C
C
Bunker
C B
CB
D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy / Q000209:etiology*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003924:Diabetes Mellitus, Type 2 / Q000139:chemically induced; D005260:Female; D015618:Histiocytosis, Sinus / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D011239:Prednisolone / Q000627:therapeutic use; D012008:Recurrence; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D011239:Prednisolone