Recurrent Purpuric Patches on the Limbs of an 18-Year-Old-Female: Gardner-Diamond Syndrome.

Sir,

An 18-year-old female patient presented with recurrent painful multiple ill-defined purpuric/ecchymotic patches on her upper and lower extremities [Figure 1]. Similar episodes had been repeated several times over the last 5 months without any history of physical trauma. Except for the skin lesions, she did not complain of any prodromal or associated symptoms. She had already been referred to the hematologist, rheumatologist and orthopediatrist with extensive investigations, all of which showed negative results or were within the normal ranges. She had been hospitalized 5 times due to recurrent painful purpuric skin lesions, which were usually resolved within 5-6 days after admission. After the second episode, she refused to go to school and finally quit school. Her symptoms and signs were a diagnostic dilemma to many clinicians. Lastly, she was referred to the Dermatology Department. She underwent skin biopsy of her skin lesions. The histopathological findings of the skin lesions showed a non-specific inflammatory reaction pattern with mild edema, erythrocyte extravasation and perivascular inflammatory cell infiltrates on the superficial dermis. At last, an autoerythrocytes sensitization test was performed. Using citrate solution as an anticoagulant, we collected the patient's peripheral blood sample. We then centrifuged the sample to isolate erythrocytes and rinsed twice with normal saline. We mixed the erythrocytes with normal saline to achieve 40% and 80% hematocrit. Intradermal injection of 0.1 ml of the achieved solution and normal saline as a control on the patient's back and forearm was performed. The patient showed positive reaction at 3 and 24 h after the autoerythrocytes sensitization test [Figure 2]. The diagnosis of Gardner-Diamond syndrome was made on the basis of clinical history complemented by the positive autoerythrocyte sensitization test. We explained the etiology of the disease and recommended referral to the psychiatric department, but she refused. However, since knowing the diagnosis of her disease, no recurrent painful purpuric patch has been observed over 8 months. Gardner-Diamond syndrome was first described by Gardner and Diamond in 1955.[1] Patients with Gardner-Diamond syndrome are known to be sensitized to their own erythrocytes, most likely phosphatidylserine, a phosphoglyceride of erythrocyte membrane.[23] Stress, personality and hormonal changes have also been considered as additional etiological factors.[4] Among the number of treatment modalities, some authors have reported positive effects of psychiatric management, but no specific treatment options have been proven to be significantly effective.[5] In the present case, without any specific psychotherapy, no recurrent purpuric patches appeared on her limbs after the diagnosis. We postulate that, without any specific therapy, just knowing the diagnosis and explaining about the nature of the disease could be helpful to the patient to prevent recurrent episodes. For recurrent painful purpuric patches over the extremities in which all investigative options have already been examined, the Gardner-Diamond syndrome should be considered as the diagnosis of exclusion.

Figure 1

Recurrent ill-defined purpuric patches on the forearm (left panel) and lower legs (right panel)

Figure 2

The autoerythrocyte sensitization test on the patient's forearm (left panel) and back (right panel) revealed the positive reaction at 3 and 24 h. (a) Normal saline; (b) autologous washed red blood cells 40%; (c) autologous washed red blood cells 80%