Psychogenic purpura.

Psychogenic purpura, also known as Gardner-Diamond syndrome, is a rare, distinctive, localized cutaneous reaction pattern mostly affecting psychologically disturbed adult women. Repeated crops of tender, ill-defined ecchymotic lesions on the extremities and external bleeding from other sites characterize the condition. We report here a case of psychogenic purpura because of the rarity of the condition and to emphasize the importance of consideration of this entity during evaluation of a patient with recurrent ecchymoses. Early diagnosis of this condition will not only minimize the cost of the medical evaluation but will also benefit the patient.

INTRODUCTION

Psychogenic purpura, also known as Gardner-Diamond syndrome, autoerythrocyte sensitization syndrome, or painful bruising syndrome, is a rare distinctive, localized cutaneous reaction pattern mostly affecting psychologically disturbed adult women. Repeated crops of bizarre, tender, ill-defined ecchymotic lesions most commonly located on the arms and legs and bleeding from other sites characterize the condition.[1] Often, there is a premonition in the form of local tingling and burning sensation a few hours prior to the appearance of the symptoms. Systemic symptoms often accompany the onset of these lesions.[2] We report here a patient diagnosed with moderate depressive episode with somatic syndrome (International Classification of Diseases (ICD)-10) who presented with psychogenic purpura because of the rarity of the condition and to emphasize the importance of consideration of this entity during evaluation of a patient with recurrent ecchymoses.

CASE REPORT

A 45-year-old woman presented with a history of recurrent painful bruise-like mark over the lower extremities for the preceding 2 years. The lesions used to appear abruptly and were heralded by tingling or burning sensation, followed by a few areas of reddish discoloration progressing to ecchymoses within a couple of days. Lesions occurred at intervals of 1-2 months. There was no itching and the lesions improved spontaneously within a week or two, imparting a bluish discoloration initially that gradually subsided without leaving any trace. There was no preceding or concomitant history of injury, of drug intake, undernutrition, external bleeding, arthralgia, respiratory infection, central nervous system, or gastrointestinal symptoms. No family history of similar illness was present. Examination revealed a few slightly tender, bluish-red, non-edematous, and ecchymotic patches of 2-5 cm in diameter over the thighs [Figure 1] and other parts of legs. Mucosae were normal and systemic examination was noncontributory. The result of intradermal autoerythrocyte sensitization test (done by injecting half ml of the patient's own blood as source of red blood cells (RBCs) into her forearm) was negative. Complete blood cell count including platelet count, bleeding time, clotting time, prothrombin time, and antinuclear antibodies were carried out on several occasions but was noncontributory. The patient was subsequently referred for psychiatric evaluation.

Figure 1

A large ecchymosis on medial aspect of left thigh

Psychiatric profile of the patient revealed that the patient was the youngest of four siblings from a poor socieo-economic background and since her childhood, she poorly expressed her feelings or emotions. At the time of presentation, she was excessively worried about her present cutaneous problem. In addition, she had low level of mood, tension-type headache, easy fatigability, poor concentration, hyper-acidity, and sleep disturbance.

Although stressors did not precede every episode, she could recall that the first episode precipitated after 4 days of her elder son's accidental death and another one was preceded by conflict with her husband regarding his extramarital relationship. She had two previous episodes of depression (the first episode, 4 years back followed by the second one 3 years ago) without any cutaneous lesion. There was no family history of any psychiatric illness. Clinical interview and mental status examination revealed moderate depressive episode with somatic syndrome (according to ICD-10). Psychometric assessment by Wechsler Adult Intelligence Scale (WAIS), Rorschach test, and Minnesota Multiphasic Personality Inventory-2 (MMPI-2) revealed that she had borderline intelligence (Full Scale Intelligence Quotient = 72) with depression, excessive interpersonal rejection sensitivity, high level of anxiety, and hypochondriacal concern. Based on the clinical course and laboratory features, the diagnosis of psychogenic purpura was made and the patient was put on escitalopram 10 mg/day and clonazepam 1 mg/day. Clonazepam was subsequently tapered off and the patient was maintained on escitalopram 10 mg/day. No recurrence of the cutaneous lesions was seen during a follow-up period of 9 months before she was lost to follow-up.

DISCUSSION

Psychogenic purpura, first described by Gardner-Diamond and named by them as autoerythrocyte sensitization (1955),[3] is a rare syndrome characterized by spontaneous, painful inflammatory ecchymoses. Because of the evidence that the occurrence of this syndrome is related to psychological factors, Ratnoff and Agle (1968) suggested that the condition be renamed “psychogenic purpura.” They postulated a psychogenic basis for the etiopathogenesis of the disorder.[4] Psychological evaluation of these patients may show hysterical and masochistic traits, depression, anxiety, and inability to deal appropriately with hostile feelings.[5] However, the precise mechanism of this syndrome is not well-understood. Our patient displayed typical ecchymoses preceded by emotional stress, had a number of systemic complaints without any objective evidence of organic disease, had depressive symptoms, and had no aberration in a battery of laboratory tests.

In addition to the cutaneous lesions, a large number of systemic symptoms have also been described in this syndrome, including abdominal pain, nausea, vomiting, joint pain, headache, and external hemorrhages such as epistaxis, gastrointestinal bleeding, and bleeding from ear canals.[6] Cutaneous responses to the intra-cutaneous injection of erythrocytes are variable.[1] Positive tests consisted of immediate itching and erythema around the injection site with the subsequent development over the next 48 hr of a typical lesion. In our patient, the response to intra-cutaneous blood was negative, but it is now obvious that evidence of autoerythrocyte sensitization is not a diagnostic prerequisite of this clinical condition, and hence, appropriately termed psychogenic purpura.[4]

The disease follows an intermittent and irregular course with variable treatment responsiveness. Treatment consists of psychiatric therapy, which is most effective when instituted early in the course of the disease; so, early diagnosis will not only minimize the cost of the medical evaluation but will also benefit the patient.[7] With the institution of regular treatment for the underlying psychological conditions, both the skin manifestations and systemic symptoms, are usually improved.