GARDNER-DIAMOND SYNDROMEMedicine Morning Report

Seuli Bose Brill, MD

3/1/10

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.

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.

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.

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.

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

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

When to consider Gardner Diamond: Recurrent ecchymoses with painful

prodrome. Normal laboratory evaluation for

bleeding diatheses. Comorbid psychiatric condition.

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.

Happy Birthday, Alan!