ARTICLE
Auteur(s) : Selda Pelin
kartal Durmazlar, Damla Atacan, Bengü Cemil, Fatma Eskioglu
Department of Dermatology, Ministry of Health Ankara
Diskapi Yildirim Beyazit Education and Research Hospital,
Ankara, Turkey
Gardner-Diamond syndrome (GDS) is a rare syndrome characterized
by spontaneous ecchymoses without definite trauma [1].
Case
A 25-year-old woman presented with a 12-year history of spontaneous
ecchymoses on her face (figure 1A). Historically,
the onset of complaints started with her menarche, related to
periods of stress and occurred at the beginning of every three or
four menstrual bleedings. Lesions were frequently preceded by
headaches and disappeared completely after a week. She had some
complaints suggesting premenstrual syndrome (PMS). Her periods were
regular with a normal flow and duration, without dysmenorrhea. She
denied any history of trauma or self injury at the site of the
lesions. She had underlying psychological problems with a difficult
parental relationship. She had attempted to commit suicide at 19,
married and divorced after 6 months and cut her arms superficially
at age 23.
On examination, she had an approximately 15 × 10 cm
ecchymotic plaque on her face. Laboratory tests, including complete
blood cell count, sedimentation rate, platelet count and
aggregation tests, were normal. Coagulation studies, including
prothrombin and partial thromboblastin time and fibrinogen,
revealed normal values. Antinuclear antibodies,
anti-double-stranded DNA, anticardiolipin antibodies, Coomb’s
direct and indirect tests and cryoglobulins were negative.
A biopsy of the lesion showed superficially extravasated
erythrocytes and a superficial infiltrate of inflammatory cells.
A diagnosis of GDS was considered. To perform the
auto-erythrocyte sensitization test, venous blood was drawn from
the patient on the 14th day of her menstrual period and collected
into tubes containing anticoagulant. The sample was centrifuged to
isolate the erythrocytes which were rinsed and then mixed with
normal saline to 70% haematocrit. Using a 25G needle, an
intradermal skin test was performed on her back with 0.1 mL
autologous erythrocytes with a saline control on the opposite side.
She was blinded to the test and under close observation. The test
caused an ecchymotic plaque with irregular borders measuring
2 cm in diameter within two hours. No lesion was observed at
the control site (figure
1B). GDS was diagnosed. We performed hormone testing which
was found to be within normal limits. We commenced 20 mg/day
fluoxetine, a selective serotonin uptake inhibitory drug,
considering its usage in PMS and the drug’s antidepressant effect.
Her skin lesions have not recurred since 1 year although the drug
was stopped by gradually tapering after 6 months. Unfortunately, we
were unable to show the hormonal influences on the onset of the
lesions as the symptoms did not recur.
Discussion
GDS usually affects adult females [1]. The ecchymoses are mostly
situated on the extremities but can, rarely, involve other areas of
the body. The ecchymoses can be replicated by subcutaneous
injection of the patient’s red cells in 59% of patients [2].
The exact mechanism of GDS is still unclear. Although GDS has
been reported in association with hematological and immunological
abnormalities, the most consistent association found in patients
with GDS is psychological problems [3, 4]. There is no specific
therapy for GDS. We postulate that antidepressant treatment with
fluoxetine, along with corrected psychosocial problems, caused the
long time remission in our patient.
To our knowledge, there is only one case in the literature where
the patient was suffering from GDS between the menarche and
menopause, where hormonal influences seem to play a role [5]. GDS
is seen in patients with mood disorders. A potential
relationship between periods of hormonal fluctuations of estrogen
and mood disorders has been suggested [6]. The fact that GDS is
mostly seen in women and our patient’s typical history led us to
consider an association between hormonal influences and GDS.
Further studies may be able to show a strong link with this
syndrome and hormonal influences.
Acknowledgements
Financial support: none. Conflict of interest: none.
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.
2 Ratnoff OD. Psychogenic purpura (autoerythrocyte
sensitization): an unsolved dilemma. Am J Med 1989; 87: 16-21.
3 Uthman IW, Moukarbel GV, Salman SM,
Taher AT, Khalil IM. Autoerythrocyte sensitization
(Gardner-Diamond) syndrome. Eur J Haematol 2000; 65: 144-7.
4 Vun YY, Muir J. Periodic painful purpura: fact or
factitious? Australas J Dermatol 2004; 45: 58-63.
5 Kirscher MH, Hofmann GO, Markewitz A,
Hatz R, Mempel M. Osteosynthetic reconstruction in a
patient with Gardner-Diamond syndrome. J Trauma 1995; 38:
392-5.
6 Halbreich U, Kahn LS. Role of estrogen in the
aetiology and treatment of mood disorders. CNS Drugs 2001; 15:
797-817.
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