Journal List > Korean J Hematol > v.42(1) > 1032761

Shin, Park, Lee, Je, Kang, and Lee: A Case of Autoerythrocyte Sensitization Syndrome in an Adult Male

Abstract

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.

REFERENCES

1). Gardner FH., Diamond LK. Autoerythrocyte sensitization: a form of purpura producing painful bruising following autosensitization to red blood cells in certain women. Blood. 1955. 10:675–90.
crossref
2). Ratnoff OD. The psychogenic purpuras: a review of autoerythrocyte sensitization, autosensitization to DNA, “hysterical” and factitial bleeding, and the religious stigmata. Semin Hematol. 1980. 17:192–213.
3). Ratnoff OD. Psychogenic purpura (autoerythrocyte sensitization): an unsolved dilemma. Am J Med. 1989. 87:16N–21N.
4). Fey MF., Beck EA. Psychogenic purpura, idiopathic thrombocytopenic purpura, and platelet dysfunction in the same patient. J Clin Psychiatry. 1986. 47:386–7.
5). Sudy E., Urbina F., Vasquez P. Autoerythrocyte sensitization syndrome with positive anticardiolipin antibodies. Br J Dermatol. 1998. 138:367–8.
crossref
6). Berman DA., Roenigk HH., Green D. Autoerythrocyte sensitization syndrome (psychogenic purpura). J Am Acad Dermatol. 1992. 27:829–32.
crossref
7). Brazzini B., Ghersetich I., Hercogova J., Lotti T. The neuro-immuno-cutaneous-endocrine network: relationship between mind and skin. Dermatol Ther. 2003. 16:123–31.
crossref
8). Ingber A., Alcalay J., Feuerman EJ. Autoerythrocyte sensitization (Gardner-Diamond syndrome) in men: a case report and review of the literature. Postgrad Med J. 1985. 61:823–6.
crossref
9). Lee H., Hann SK. Autoerythrocyte sensitizaton syndo-rme: a case report. Korean J Dermatol. 1999. 37:1355–7.
10). Lee GC., Kim YG., Kim MK., Yoon TY. Autoerythrocyte sensitization syndrome. Ann Dermatol. 2005. 17:27–9.
crossref
11). Tomec RJ., Walsh M., Garcia JC., Jordan PK. Diagnosis of autoerythrocyte sensitization syndrome in the emergency department. Ann Emerg Med. 1989. 18:780–2.
crossref
12). Vun YY., Muir J. Periodic painful purpura: fact or factitious? Australas J Dermatol. 2004. 45:58–63.
crossref
13). 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. 2006. 72:379–81.
14). Spiera H., Schwartz AL. Autoerythrocyte sensitization reproductible by both autologous red cells and heterologous DNA. Mt Sinai J Med. 1970. 37:108–11.
15). Anderson JE., DeGoff W., McNamara M. Autoerythrocyte sensitization (psychogenic purpura): a case reoport and reivew of the literature. Pediatr Emerg Care. 1999. 15:47–8.
16). Settle EC Jr. Autoerythrocyte sensitization successfully treated with antidepressants. JAMA. 1983. 250:1749–50.
crossref
17). Utman IW., Moukarbel GV., Salman SM., Salem ZM., Taher AT., Khalil IM. Autoerythrocyte sensitization (Gardner-Diamond) syndrome. Eur J Haematol. 2000. 65:144–7.

Fig. 1
Spontaneously developed ecchymoses over the lower leg.
kjh-42-48f1.tif
Fig. 2
Autoerythrocyte sensitization test at 4th day.
kjh-42-48f2.tif
Table 1.
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
Table 2.
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.

Table 3.
Agents associated with positive intradermal res ponse
Copper
Erythrocyte stroma
Hemoglobin
Histamine
Phosphatidylserine
Platelets
Pregnandiol
Purified protein derivative
Serotonin
Tyramine

From reference 12.

Table 4.
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.

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