Gardner–Diamond syndrome complicated with chronic urticaria successfully treated with antihistamine

Gardner–Diamond syndrome (GDS) is a rare condition characterized by painful ecchymotic lesions in the extremities which usually occur following emotional stress; thus, GDS is considered a form of psychogenic purpura.1, 2 GDS is also known as autoerythrocyte sensitization syndrome; however, the pathophysiology of this condition has yet to be fully understood.3 Herein, we present a case of GDS, in which the ecchymosis was suppressed by antihistamine administration. A 19-year-old female patient presented with ecchymoses in the extremities of 3 months’ duration. On presentation, she was generally healthy with no past psychiatric or medical history. However, she mentioned a stressful situation she experienced at university and was concerned about the seriousness of her skin eruptions. Dermatological examination revealed several palm-sized ecchymoses in the extremities (Figure 1a). Immediately prior to their development, the patient reported experiencing a subtle burning sensation at the lesion sites. She also complained of skin itch associated with erythema. She noticed itchy eruption, which rapidly disappeared, for several months. Itch and erythema occurred anywhere in her body; however, the relationship between ecchymoses and itchy eruption was unclear. Although the itchy eruption could not be detected at the medical examination, dermatographia was observed (Figure 1b). All laboratory examination results, including a blood coagulation test and platelet count, were normal. An i.d. injection of the patient’s own red blood cells induced a recurrence of the ecchymosis, but a saline injection had no effect (Figure 1c). Based on the clinical findings and medical examination results, GDS with chronic urticaria was diagnosed. The patient was informed that her condition was not serious and was given a prescription of bepotastine besilate 10 mg b.i.d. for the urticaria, which responded well to the treatment. Interestingly, the ecchymotic lesions seldom relapsed after antihistamine use. Gardner–Diamond syndrome is a rare cutaneous disorder, the definitive diagnosis of which is challenging. The autologous erythrocyte sensitization skin test has historically been used for the diagnosis for GDS and this test was positive in the patient in this case. However, dermatologists should keep in mind that the usefulness of this test is now controversial due to the lack of data on its efficacy. The treatment for GDS remains challenging due to the small number of cases and inadequate understanding of its pathophysiology. Treatments reportedly effective for the condition include selective serotonin reuptake inhibitors, corticosteroids, and tricyclic antidepressants, but the effects of these drugs are inconsistent.1 Previous studies have reported cases of GDS treated with antihistamines and psychiatric therapy; however, the therapeutic efficacy was various.1, 4, 5 In the present case, the patient received antihistamines for her chronic urticaria. Interestingly, the ecchymoses as well as urticaria resolved after antihistamine administration alone. Urticaria is usually caused by emotional stress; thus, the coincidence of GDS and urticaria may not be infrequent. The mechanism underlying individual differences in response to antihistamine remains unclear. In the present case, scratching due to urticaria, which was inhibited by antihistamine, might have induced the ecchymoses. In other words, mechanical stress might trigger this symptom of GDS. None. None declared.