Gardner-Diamond syndrome is a rare clinical entity characterized by the spontaneous development of bruising and subsequent skin symptoms. An autoimmune reaction to phosphatidylserine in erythrocytes triggered by significant emotional stress or physical trauma seems to be the mechanism of disease. The diagnosis is made by injection of the patient's own red blood cells into the dermis and observation of an autoimmune reaction.
The clinical presentation of patients suffering from Gardner-Diamond syndrome (also known as autoerythrocyte sensitization syndrome) seems to start with physical trauma of the skin, seen in surgery, blunt force or accidental injuries, while emotional trauma - abortion, divorce, anxiety, etc. are also presumable triggers  . In fact, the term psychogenic purpura is used to describe typical changes seen in this condition  . The cause still remains undisclosed, but patients initially develop constitutional symptoms such as malaise and fatigue, followed by a burning, itching or stinging sensation, pain and induration of the skin with bruising   . As the lesions grow into pink or red plaques of various sizes and progress to bluish ecchymoses, a misdiagnosis for cellulitis or compartment syndrome is not uncommon, but spontaneous regression and complete resolution within one week and subsequent recurrence is the typical clinical course of lesions encountered in this condition . Skin lesions may be encountered at any site in the body, but the most common localization are the extremities . They are described in individuals of all ages, including children, and about 200 cases are documented in the literature, suggesting its very rare occurrence in medical practice  . In addition, numerous systems may be affected, including gastrointestinal (epigastric pain, nausea, vomiting, diarrhea) and vascular (subconjunctival hemorrhage, epistaxis, hematuria, menorrhagia, bleeding from the ear canal), while glomerulonephritis, stroke, and lymphoid abnormalities are extremely rare but observed findings  . Most common psychiatric disorders encountered in these patients include conversion symptoms, hysteria, depression, anxiety, and dissociative reactions  .
Entire Body System
A 34-year-old woman with a 3-year history of easy bruising of unexplained aetiology in anatomical sites consistent with self-infliction is described. Despite major surgery, the patient had never experienced inappropriate bleeding. [ncbi.nlm.nih.gov]
A 24-year-old man with a 10-year history of easy bruising of undefined aetiology in anatomical sites consistent with self-infliction is reported. [tandfonline.com]
She did not have a history of abnormal bleeding or easy bruising. There was no menorrhagia, hematuria or dysuria. Recent wisdom teeth extraction was not complicated by excessive bleeding. [academic.oup.com]
Abstract The case of a patient with two unusual dermatologic entities, multiple glomus tumors and the painful purpura of Gardner-Diamond syndrome, is reported. [ncbi.nlm.nih.gov]
Psychogenic purpura (autoerythrocyte sensitization): an unsolved dilemma. Am J Med 1989;87:16N–21N. 4. Fey MF, Beck EA. Psychogenic purpura, idiopathic thrombocytopenic purpura, and platelet dysfunction in the same patient. [synapse.koreamed.org]
Psychogenic purpura (autoerythrocyte sensitization) South Med J. 1981;74:538-42. Yücel B, Kiziltan E, Aktan M. Dissociative identity disorder presenting with psychogenic purpura. Psychosomatics. 2000;41:279-81. [sci-rep.com]
A form of purpura producing painful bruising following autosensitization to red blood cells in certain women. Blood. 1955;10:675-90. Ratnoff OD. Psychogenic purpura (autoerythrocyte sensitization): an unsolved dilemma. Am J Med. 1989;87:16-21. [ijmedicine.com]
Diamond, American physician, b. 1902 a condition resulting from autoerythrocyte sensitization, marked by large, painful transient skin discolorations that appear without apparent cause but often accompany emotional upsets, various collagen disorders, [medical-dictionary.thefreedictionary.com]
Most common psychiatric disorders encountered in these patients include conversion symptoms, hysteria, depression, anxiety, and dissociative reactions. [symptoma.com]
Histrionic Personality Disorder
personality disorder, as well as sadomasochistic relationships, with frank abuse by significant others. [ijpm.info]
To make the diagnosis, it is necessary to conduct a thorough clinical and laboratory workup that will exclude other more common conditions presenting with similar symptoms. Firstly, a detailed patient history is necessary, which will identify information regarding the onset and duration of symptoms, family history of bleeding disorders and detection of possible events that induce a significant amount of stress . A meticulous physical examination should follow, and complete inspection of the skin can reveal numerous ecchymoses (either resolving or evolving) on different parts of the body . Additionally, exclusion of puncture wounds (seen in IV drug users) as the cause of skin lesions must be made   . Laboratory workup with levels of hemoglobin, hematocrit, platelet count and erythrocyte sedimentation rate within physiological levels, while prothrombin time, activated partial thromboplastin time, as well as inflammatory markers, serum electrolytes, and specific coagulation factors will also be normal . These findings will exclude many important disorders (disseminated intravascular coagulation, Henoch-Schonlein purpura, von Willebrand disease, systemic lupus erythematosus, infection, etc.) but a definite diagnosis can be obtained by injecting patient's own washed erythrocytes into the dermis, and a subsequent autoimmune reaction (to phosphatidylserine in their stroma) will cause bruising  . In addition, histopathological analysis can be performed by obtaining a sample of tissue. Extravasation of blood cells, as well as the absence of inflammatory or infectious components, is always encountered  .
Normocytic Normochromic Anemia
Diamond, Louis Klein, U.S. physician, 1902-1999 Diamond-Blackfan anemia - autosomal recessive normocytic normochromic anemia. Synonym(s): congenital hypoplastic anemia ; Diamond-Blackfan syndrome Gardner, Frank H., U.S. hematologist, 1919–. [medical-dictionary.thefreedictionary.com]
Punch biopsies of the skin lesions revealed scant superficial perivenular lymphocytic infiltration. [apallergy.org]
A skin biopsy of the bruise showed nonspecific lymphocytic infiltration and hemorrhage. There was no evidence of vasculitis, and immunofluorescence studies were negative. An upper endoscopy demonstrated esophagitis, but was otherwise normal. [academic.oup.com]
Due to the rare nature of this disorder, there exist few guidelines for prompt clinical diagnosis and optimal treatment. [alliedacademies.org]
Dermatological signs identical to Gardner-Diamond syndrome should alert the physician to avoid unnecessary fasciotomy, as these patients respond well to psychiatric treatment as in the present case. [ncbi.nlm.nih.gov]
Treatment Options No controlled studies exist. Treatment should be directed toward lesion control, pain control, and intervention for an underlying or associated psychiatric disorder. [dermatologyadvisor.com]
Prognosis - Gardner-Diamond Syndrome Not supplied. [checkorphan.org]
The prognosis of AES syndrome is good; no fatalities have been reported secondary to the disease. However, the lesions have recurred for as long as 30-40 years. [apallergy.org]
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). [dermatologyadvisor.com]
16 Unfortunately, in the majority of patients reported with Gardner-Diamond syndrome, the results of the tests for a primary hemostatic disorder are not available; therefore, its prevalence in this population of patients is completely unknown.17 The prognosis [elsevier.es]
Owing to the uncertain etiology, we suggest that the functional platelet defect found may intervene, maintaining and making the ecchymotic lesions worse. [ncbi.nlm.nih.gov]
Etiology The etiology is unclear. Stress and psychiatric disorders frequently are present; possibly etiologic. [dermatologyadvisor.com]
Subsequent investigations failed to elucidate an organic etiology. [academic.oup.com]
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. [e-ijd.org]
(level of evidence 2B) ALLEN, G., et al., Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients. Pain,1999. [physio-pedia.com]
The pathophysiology of the disease is not fully understood, and little is known about management modalities for this syndrome. [read.qxmd.com]
Brenner/Rector remains the go-to resource for practicing and training nephrologists and internists who wish to master basic science, pathophysiology, and clinical best practices. [books.google.com]
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 [dermatologyadvisor.com]
Though our study provides new insights regarding dx, therapy and LTO, future work investigating the pathophysiology leading to the hemostatic abnormalities in GDS would be valuable. [bloodjournal.org]
Prevention - Gardner-Diamond Syndrome Not supplied. Diagnosis - Gardner-Diamond Syndrome Not supplied. Prognosis - Gardner-Diamond Syndrome Not supplied. [checkorphan.org]
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. [e-ijd.org]
Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive [jamanetwork.com]
Author Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public [emedicine.medscape.com]
"Force majeure" term will be regarded as outside of the concerned party's reasonable control and any situation that "Turkiye Klinikleri" cannot prevent even though it shows due diligence. [turkiyeklinikleri.com]
- Jafferany M, Bhattacharya G. Psychogenic Purpura (Gardner-Diamond Syndrome). Prim Care Companion CNS Disord. 2015;17(1):10.4088/PCC.14br01697.
- Zhao H, Luo F, Li H. Autoerythrocyte Sensitization Syndrome Presenting with General Neurodermatitis: Factitious Purpura or Psychophysiological Entity? Dermatol Ther (Heidelb). 2012;2(1):5
- Meeder R, Bannister S. Gardner-Diamond syndrome: Difficulties in the management of patients with unexplained medical symptoms. Paediatr Child Health. 2006;11(7):416-419.
- Okur M, Turan H, Özkan A, Güneş C, Kocabay K. An Extremely Rare Cause of Bruising in Children: Autoerythrocyte Sensitization Syndrome. Turk J Haematol. 2012;29(2):201-203.
- Sarkar S, Ghosh SK, Bandyopadhyay D, Nath S. Psychogenic purpura. Indian J Psychiatry. 2013;55(2):192-194.