kjh The Korean Journal of Hematology Korean J Hematol 1738-7949 2092-9129 Korean Society of Hematology 10.5045/kjh.2007.42.1.48 kjh-42-48 Case Report A Case of Autoerythrocyte Sensitization Syndrome in an Adult Male ShinHo SikM.D. 1 ParkChan HoM.D. 1 LeeChan WooM.D. 2 JeHo JunM.D. 3 KangHun DaeM.D. 4 LeeSang HarkM.D. 5 Department of Internal Medicine, Busan, Korea Department of Dermatology, Busan, Korea Department of Psychiatry, Busan, Korea Department of Surgery, Busan, Korea Department of Orthopedic Surgery, The Armed Forces Busan Hospital, Busan, Korea Correspondence to：Ho Sik Shin, M.D. Department of Internal Medicine, The Armed Forces Busan Hospital San 47-3, Jwa-dong, Haeundae-gu, Busan 612-030, Korea Tel: +82-51-701-9147, Fax: +82-5 1-703-9147 E-mail: danieljoseph@hanmail.net 032007 19032007 42148 52 28112006 11122006 03012007 Copyright © 2007 Korean Society of Hematology 2007 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Autoerythrocyte sensitization syndrome (Gardner-Diamond syndrome), also known as painful bruising syndrome or psychogenic purpura, is characterized by sudden and spontaneous painful bruising and swelling on both extremities, and is a psychologically induced painful bruising condition. The exact etiology of the syndrome is an enigma, even today. It occurs primarily in young to middle-aged women who usually have psychogenic disorders. A diagnosis of this syndrome is made on the basis of the clinical history, dermatological examination complemented by a positive autoerythrocyte sensitization test, psychiatric evaluation and the absence of any organic cause. We present a 20-year-old man with characteristic features of this syndrome, who had a positive autoerythrocyte sensitization test. To our knowledge, this is the first case in an adult male reported in the Korean literature.

 Autoerythrocyte sensitization syndrome Gardner-Diamond syndrome Painful bruising syndrome Psychogenic purpura REFERENCESGardner FH Diamond LK Autoerythrocyte sensitization: a form of purpura producing painful bruising following autosensitization to red blood cells in certain women Blood 195510 67590 Ratnoff OD The psychogenic purpuras: a review of autoerythrocyte sensitization, autosensitization to DNA, “hysterical” and factitial bleeding, and the religious stigmata Semin Hematol 198017 192213 Ratnoff OD Psychogenic purpura (autoerythrocyte sensitization): an unsolved dilemma Am J Med 198987 16N21N Fey MF Beck EA Psychogenic purpura, idiopathic thrombocytopenic purpura, and platelet dysfunction in the same patient J Clin Psychiatry 198647 3867 Sudy E Urbina F Vasquez P Autoerythrocyte sensitization syndrome with positive anticardiolipin antibodies Br J Dermatol 1998138 3678 Berman DA Roenigk HH Green D Autoerythrocyte sensitization syndrome (psychogenic purpura) J Am Acad Dermatol 199227 82932 Brazzini B Ghersetich I Hercogova J Lotti T The neuro-immuno-cutaneous-endocrine network: relationship between mind and skin Dermatol Ther 200316 12331 Ingber A Alcalay J Feuerman EJ Autoerythrocyte sensitization (Gardner-Diamond syndrome) in men: a case report and review of the literature Postgrad Med J 198561 8236 Lee H Hann SK Autoerythrocyte sensitizaton syndo-rme: a case report Korean J Dermatol 199937 13557 Lee GC Kim YG Kim MK Yoon TY Autoerythrocyte sensitization syndrome Ann Dermatol 200517 279 Tomec RJ Walsh M Garcia JC Jordan PK Diagnosis of autoerythrocyte sensitization syndrome in the emergency department Ann Emerg Med 198918 7802 Vun YY Muir J Periodic painful purpura: fact or factitious? Australas J Dermatol 200445 5863 Sawhney MP Arora G Arora S Prakash J Undiagnosed purpura: a case of autoerythrocyte sensitization syndrome associated with dermatitis artefacta and psedo-ainhum Indian J Dermatol Venereol Leprol 200672 37981 Spiera H Schwartz AL Autoerythrocyte sensitization reproductible by both autologous red cells and heterologous DNA Mt Sinai J Med 197037 10811 Anderson JE DeGoff W McNamara M Autoerythrocyte sensitization (psychogenic purpura): a case reoport and reivew of the literature Pediatr Emerg Care 199915 478 Settle EC Jr Autoerythrocyte sensitization successfully treated with antidepressants JAMA 1983250 174950 Utman IW Moukarbel GV Salman SM Salem ZM Taher AT Khalil IM Autoerythrocyte sensitization (Gardner-Diamond) syndrome Eur J Haematol 200065 1447 Figures and Tables

Spontaneously developed ecchymoses over the lower leg.

Autoerythrocyte sensitization test at 4th day.

Results of investigation

Investigation Results Reference range
Full blood count Normal －
Activated partial thromboplastin time 40.1 25～42 sec
Prothrombin time 13.4 11～16 sec
Fibrinogen 3.7 1.5～4.0g/L
Platelets 184 150～400×109/L
Factors VIII, IX, XI Normal －
Factors XII 0.56 0.5～1.5U/mL
Von Willebrand factor Normal －
Complement factor C3 1.50 0.9～2.0g/L
Complement factor C4 0.30 0.15～0.45g/L
Cryoglobulin Not detected －
Antinuclear antibodies Negative －
Double-stranded DNA antibodies ＜5 ＜7IU/mL
Lupus anticoagulant Negative －
Anticardiolipin immunoglobulin G 4 ＜8U/mL

Commonly associated complaints in autoerythro cyte sensitization syndrome

Neurological symptoms
    Headaches
    Transient paresis
    Paresthesia
    Syncopal episode
Ocular symptoms
    Blurred vision
    Diplopia
Hemorrhagic manifestation
    Epistaxis
    Gastrointestinal bleeding
Abdominal pains
Chest pains
Muscular and joint aches
Vague drug-related urticaria or rashes

From reference 12.

Agents associated with positive intradermal res ponse

Copper
Erythrocyte stroma
Hemoglobin
Histamine
Phosphatidylserine
Platelets
Pregnandiol
Purified protein derivative
Serotonin
Tyramine

From reference 12.

Reported treatments for autoerythrocyte sensitization syndrome

6-mercaptopurine
Albumin infusion
Antibiotics
Anticoagulants
Antihistamines
Busulphan
Chloroquine
Corticosteroids (prednosolone, hydrocortisone)
Cyproheptadine
Desensitization with red cell extracts/stroma
Hormones (estrogens, progestagens)
Immunosuppressive agents
Meperidine
Pentoxifylline
Plasmapheresis
Psychotherapy and antidepressants
Splenectomy
Vitamin C

From reference 12.