Dermatology

Psychogenic Purpura (Gardner-Diamond syndrome, autoerythrocyte sensitization syndrome, painful bruising syndrome)

Psychogenic Purpura also known as Gardner-Diamond syndrome, autoerythrocyte sensitization syndrome, painful bruising syndrome. 287.2 (Other nonthrombocytopenic purpuras)

Are You Confident of the Diagnosis?

Rule out factitious, malingering, and abuse.

What you should be alert for in the history

Be alert for predictable bruising associated with psychological stress in the absence of a hematologic, vascular, immunologic, or infectious process.

Characteristic findings on physical examination

Characteristic findings include unexplained painful, ecchymotic lesions, mostly on the extremities and the face. Initial symptoms include burning and pain followed by erythema, edema, pruritus, and eventually ecchymoses. Lesions may be accompanied by systemic symptoms; gastrointestinal, rheumatologic, neurologic. Lesions are often precipitated by emotional stress, and may occur after surgery or trauma. Patients often present with a psychiatric comorbidity.

Expected results of diagnostic studies

Hematologic and coagulation studies are normal. Obtain a complete blood count (CBC) with differential, platelet count, platelet function analyzer (PFA-100), antiphospholipid antibodies, von Willebrand’s panel, antinuclear antibody (ANA), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) to rule out other organic etiology. The autoerythrocyte sensitization test (0.1ml intradermal injection of washed autologous erythrocytes) is unreliable and generally not useful.

Diagnosis confirmation

It is important to rule out coagulopathy, vasculitis, von Willebrand’sdisease, Ehlers-Danlos, hereditary hemorrhagic telangiectasia,Henoch-Schönlein purpura, vitamin C deficiency, antiphospholipidsyndrome, medications (acetylsalicylic acid, nonsteroidal antiinflammatory drugs, glucocorticoids, possibly selective serotonin reuptake inhibitors (SSRIs),and illicit drugs.

Who is at Risk for Developing this Disease?

Middle-aged Caucasian females with psychiatric comorbidity appear at greatest risk. Pediatric and male cases have been reported.

What is the Cause of the Disease?

Etiology

The etiology is unclear. Stress and psychiatric disorders frequently are present; possibly etiologic.

Pathophysiology

Postulated mechanisms include a stress-induced increase in endogenous glucocorticoids, causing altered hemostasis, increased fibrinolysis secondary to increased activity of tissue plasminogen activator, and autoerythrocyte sensitization (autosensitization to phosphoglyceride on the RBC membrane). Malingering, factitious disorders are possible; but one must must first rule out organic etiologies. Rule out relationship abuse.

Systemic Implications and Complications

Affected patients tend to have many associated somatic complaints and psychiatric comorbidities. No clear link established between disorder itself and other pathologic biologic entities.

Treatment Options

No controlled studies exist. Treatment should be directed toward lesion control, pain control, and intervention for an underlying or associated psychiatric disorder. Psychotherapy should be suggested as the most effective intervention.

Medical treatment includes:

Bland topicals; ie, petrolatum or Aquaphor, for open lesions.

Topical lidocaine 2-5% cream or gel, topical Doxepin 5% cream for pain control.

Amitryptyline 10-50mg at night or twice daily dosing for pain control.

Consider gabapentin 100mg three times a day, titrating to 300mg three times a day, for pain control.

SSRIs and selective norepinephrine reuptake inhibitors (SNRIs) may help associated obsessive-compulsive disorder (OCD) and depressive symptoms. Caution is warranted since there are scattered case reports of bruising with SSRI’s. There are anecdotal suggestions for use of of beta blockers, on the grounds of their efficacy for migraine headaches and reflex sympathetic dystrophy.

Strongly recommend psychotherapy. Suggest to the patient that psychotherapy can diminish the occurrence of the disease and help them deal with the capricious, painful, and cosmetically visible nature of the disease.

Optimal Therapeutic Approach for this Disease

Once confident in the diagnosis, reassure the patient that the prognosis is good. Explain that most will have remission of symptoms (except possible recurrences during stressful periods). Help the patient understand the potential etiologic role of stress as an aggravating factor. This is the rationale for psychiatric referral. Strongly suggest and help facilitate a psychiatric referral, if possible.

Introduce topical and oral agents along with psychotherapy until lesions and symptoms abate. Observe for signs of more severe psychopathology, underlying organic disease, drug use or abuse, physical or emotional abuse.

Patient Management

Periodically repeat a thorough review of systems and appropriate laboratory studies.

Unusual Clinical Scenarios to Consider in Patient Management

Be vigilant for purposely inflicted factitious lesions and malingering for secondary gain.

What is the Evidence?

Yücel, B, Kiziltan, E, Aktan, M. "Dissociative identity disorder presenting with psychogenic purpura". Psychosomatics. vol. 41. 2000. pp. 279-81.

(Brief review of psychogenic purpura and case report suggesting entity may be a dissociative identity disorder associated with abuse.)

Boussault, P, Doutre, MS, Beylot-Barry, M. "Painful bruising syndrome: a psychogenic disease". Rev Med Interne. vol. 26. 2005. pp. 744-7.

(Provides hypotheses regarding the etiology with recommendations for treatment, including beta blockers.)

Meeder, R, Bannister, S. "Gardner-Diamond syndrome: difficulties in the management of patients with unexplained medical symptoms". Paediatr Child Health. vol. 11. 2006. pp. 416-9.

(Documents the occurrence of Gardner-Diamond syndrome in the pediatric population.)

Siddi, GM, Montesu, MA. "Gardner-Diamond syndrome". JEADV. vol. 20. 2006. pp. 735-7.

(Good overview of syndrome with presenting symptoms and treatment recommendations.)

Uthman, IW, Moukarbel, GV, Salman, SM. "Autoerythrocyte sensitization (Gardner-Diamond) syndrome". Eur J Haematol. vol. 65. 2000. pp. 144-7.

(Case report of patient with Gardner-Diamond syndrome supporting abnormaility in platelet aggregation studies.)

Sotiriou, E, Apalla, Z, Apalla, K, Panagiotidou, D. "Case Report: Psychogenic purpura". Psychosomatics. vol. 51. 2010;May-June. pp. 274-5.

(Case report and succinct overview of entity and treatment.)
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