Gardner-Diamond Syndrome

1. Gardner-Diamond Syndrome Medicine Morning Report Seuli Bose Brill, MD 3/1/10

2. Background • Described in 1955 by Frank Gardner and Louis Diamond. • Original case report described painful ecchymoses in 4 female patients. • Other names include: • Psychogenic purpura • Autoerythrocyte sensitization • Painful bruising syndrome • About 120 cases described in literature to date.

4. Presentation • Unexplained painful ecchymotic lesions. • Involves extremities or the face. • Caucasian women. • Precipitated by emotional stress. • Spontaneous bruising after trauma or surgery. • May occur after a serious emotional disturbance. • Affected individual generally with psychiatric comorbidities.

5. Pathophysiology • Stress induced • Increased levels of endogenous glucocorticoids alters hemostasis. • Increased fibrinolysis • Increased cutaneous activity of tissue plasminogen activator. • Autoerythrocyte sensitization • Autosensitization to phosphoglyceride on RBC membrane.

6. Diagnosis • Clinical. • Laboratory tests generally negative. • PT, PTT, platelet count, platelet function analyzer (PFA-100), anti-phospholipid antibodies, Von Willebrand’s panel, ANA, ESR, CRP. • Auto-erythrocyte sensitization test not useful. • Not standardized or commercially available. • Histopathological exam not necessary.

8. Differential Diagnosis • Malingering and factitious disorders • Von Willebrand’s disease • Vitamin C deficiency • Small vessel vasculitis • Anti-phospholipid syndrome • Drugs • Ecstasy use • ASA, NSAIDS • SSRIs • Systemic glucocorticoids • Ehlers-Danlos • Hereditary hemorrhagic telangiectasia • Henoch-Schonlein purpura

9. Treatment • Plasmapheresis • No clinical trials • Case report in which plasmapheresis vs. placebo offered to patient who was blinded to therapy. • Pain control • Amitryptyline • Promethazine • Treatment of underlying psychiatric disorder

10. When to consider Gardner Diamond: • Recurrent ecchymoses with painful prodrome. • Normal laboratory evaluation for bleeding diatheses. • Comorbid psychiatric condition.

11. References • Bostwick JM, Imig MW. Gardner-Diamond Syndrome: bruising feeling. Mayo Clin Proc. 2008 May;83(5):572. • Gardner, F. and Diamond, L. Autoerythrocyte sensitization, A Form of Purpura Producing Painful Bruising Following Autosensitization to Red Blood Cells in Certain Women. Blood. 1955. 10(7): 1955-1971. • Giesler, B and Dezube, B. Psychogenic Purpura (Gardner-Diamond Syndrome). UpTo Date. April 15,2009. • Hamblin, T, Hart, S, Mufti, GJ. Plasmapheresis and a placebo procedure in autoerythrocyte sensitization. British Medical Journal. 1981 Dec: 283: 1575-1576. • Ivanov OL, Lvov AN, Michenko AV, Künzel J, Mayser P, Gieler U. Autoerythrocyte sensitization syndrome (Gardner-Diamond syndrome): review of the literature. J Eur Acad Dermatol Venereol. 2009 May;23(5):499-504. • Meeder R, Bannister S. Gardner-Diamond syndrome: Difficulties in the management of patients with unexplained medical symptoms. Paediatr Child Health. 2006 Sep;11(7):416-9. • Uthman IW, Moukarbel GV, Salman SM, Salem ZM, Taher AT, Khalil IM. Autoerythrocyte sensitization (Gardner-Diamond) syndrome. Eur J Haematol. 2000 Aug;65(2):144-7.

12. Happy Birthday, Alan!