Gardner diamond syndrome: A psychogenic purpura.

A 35-year-old housewife presented with recurrent episodes of spontaneously appearing ecchymotic patches over lower extremities associated with pain and burning sensation of 6-months duration.[Figure 1]. Lesions resolve in 7–10 days leaving temporary hyperpigmentation. There was no prior history of trauma, menstrual irregularities, bleeding diathesis, any significant drug or family history. Other clinical examination was unremarkable. Complete blood cell count, coagulation profile and antinuclear antibodies were noncontributory. Intradermal injection of 0.1 ml autologous washed erythrocytes on flexure aspect of the left forearm caused an ecchymotic patch of approximately 2cm diameter in 2 hours [Figure 2]. Based on the above findings a final diagnosis of Gardner–Diamond syndrome was made.

Figure 1

Ill-defined ecchymotic patch of 2-5 cm size over the left thigh

Figure 2

Positive auto-erythrocyte sensitization test compared to control (normal saline) over the left forearm

Psychiatric consultation revealed symptoms suggestive of mixed anxiety depression disorder. Treatment was initiated with tablet Amytriptyline 10 mg and Clonazepam 0.5 mg. There was no further new episodes during a follow-up period of 6 months.

Auto-erythrocyte sensitization syndrome, also known as Gardner–Diamond syndrome (painful bruising syndrome or psychogenic purpura), is a rare disorder first described by Gardner and Diamond in 1955.[1] It typically occurs in middle-aged females with underlying psychiatric disorders such as depression, anxiety or obsessive compulsive disorders. Autosensitization of patients to their own blood, mainly to phosphatidylserine [phosphoglyceride of red blood cell (RBC) membrane] plays an important role in the pathogenesis.[2]

It is characterized by recurrent self-limiting episodes of spontaneously appearing painful ecchymoses, frequently preceded by a prodrome of burning sensation of the skin.[3] There are no specific laboratory findings. An intracutaneous injection of 0.1 ml of autologous washed erythrocytes is a reliable diagnostic test.[2]

A recent hypothesis proposed by researchers of psychoimmunology states the intricacies of the neuroimmunocutaneous endocrine network, which might explain the response of anxiolytics and antidepressants in our case. Sometimes psychotherapy may be beneficial.[4]

Though it is a rare disease, it should be considered in the differential diagnosis of recurrent painful purpura. Prompt diagnosis helps to avoid exhaustive investigations and aggressive treatment.

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