Diagnosing the Dermatologic Blues: Systematic Review of the Rare Conundrum, Psychogenic Purpura.

BACKGROUND: Psychogenic purpura is an idiopathic psychodermatologic condition of recurrent, painful purpura precipitated by psychological stress, predominantly affecting young females. Little consensus exists on the diagnostic guidelines for this rare condition, often resulting in costly, unnecessary, and stressful investigations as well as prolonged hospital admissions. OBJECTIVE: With this first up-to-date systematic review of 134 cases of psychogenic purpura in over a decade, we aim to thoroughly investigate the diagnostic strategy and treatment regimens used in the last decade. With a sooner diagnosis, patient stress and nosocomial ecchymoses can be minimized, and treatment can be expedited. METHODS: We conducted a literature review of 4 databases (PubMed, Ovid Embase, Ovid MEDLINE, and Web of Science) on October 5, 2022 that yielded 46 full-text articles, which were reviewed and extracted by 2 independent reviewers. RESULTS: We analyzed a total of 134 cases, consisting largely of females (125/134, 93.3%) with purpura on the upper (103/134, 76.9%) or lower limbs (112/134, 83.6%). Apart from a paresthesia prodrome, patients commonly experienced headaches, malaise, and arthralgia or myalgia. Approximately 70% (95/134) of patients reported a physiological or psychological stressor or psychiatric diagnosis before the development of the purpura. Laboratory testing almost always revealed unremarkable results. The intradermal washed autoerythrocyte sensitization test was positive in 98% (42/43) of cases. Histopathology biopsy findings commonly revealed dermal erythrodiapedesis or hemorrhage (n=34) and perivascular inflammatory infiltrates (n=17). Approximately 42% (56/134) of patients received a novel psychiatric diagnosis, with depression being the most common (40/72, 56%). In both patients with and those without a novel psychiatric diagnosis, observation, counseling, treatment with antidepressants (ie, selective serotonin reuptake inhibitors), and psychotherapy (ie, cognitive behavioral therapy) prevailed in the resolution of the purpura. CONCLUSIONS: Due to the unclear etiology and infrequent presentation of this condition, it remains a diagnosis of exclusion based on clinical suspicion evaluating the presence of stressors or psychiatric comorbidities and exclusion of systemic conditions. Clinical confirmation can be sought through a positive autoerythrocyte sedimentation test, characteristic histopathology findings, and remission of purpura after psychiatric treatment.

Is Gardner-Diamond syndrome related to autoimmunity?

Gardner and Diamond described a clinical picture of painful ecchymosis in the skin and mucous membranes in four female patients and called this entity Gardner-Diamond syndrome (GDS). At present, the exact pathogenesis of the disease is still unknown. In recent years, it has been advocated that antibodies against phosphatidylserine in erythrocyte stroma may cause immune complex and complement activation, leading to this clinical picture. Herein, we found it appropriate to present a case of multiple ecchymotic lesions diagnosed with GDS followed by many autoimmune diseases to draw attention to autoimmune association. As a result of this case presentation and mini-literature review, we think that autoimmunity patients should not be missed in GDS patients.

Oral management in a patient with Gardner-Diamond Syndrome: A case report.

INTRODUCTION: Gardner-Diamond Syndrome (GDS) is rare. It is characterized by the spontaneous formation of painful erythematous skin lesions that develop into ecchymoses within 24 h and then disappear progressively over days to weeks. The complications can be serious. PRESENTATION OF CASE: A 35-year-old man with GDS was admitted to the oral surgery department for dental infectious focus eradication. Clinical and radiological examinations indicated extraction of tooth 17. It was performed with local anaesthesia, cardiac monitoring, and verbal reassurance therapy. After delivering anaesthesia, two intra-oral hematomas and a bruise quickly developed. Cardiovascular manifestations and a spontaneous painful right temporal erythematous skin lesion appeared in the next 24 h. The patient was briefly hospitalized in the cardiovascular medicine department. Over the next 21 days, some haematomas regressed, another expanded, and a new cervico-thoracic ecchymosis developed. DISCUSSION: The surgical and post-surgical complications in this clinical case raise several points concerning the oral management of patients with GDS. We propose to apply: verbal reassurance therapy during surgery; presurgical haematological evaluation and postsurgical daily haematological follow-up; and atraumatic surgery with intra- and post-surgical haemostatic precautions. Systematic antibiotic prophylaxis and non-steroidal anti-inflammatory drugs may also be useful. Other possibilities include desmopressin acetate, corticosteroids, antihistamines, plasmapheresis, and immunosuppressive agents; however, few cases have been treated with these strategies. CONCLUSION: This case highlights the difficult management of patients with GDS who require oral surgery. Further studies are needed to improve the oral surgical procedures in these patients and to establish a systematic management algorithm.

Spontaneous Ecchymotic Lesions with Systemic Symptoms: A Quiz.

is missing (Quiz).

Síndrome de Gardner-Diamond. Presentación de un caso / Gardner-Diamond syndrome: A case report

RESUMEN El sindrome de Gardner- Diamond conocido también como púrpura psicógena o síndrome de autosensibilización eritrocitaria es muy poco frecuente. Se presenta el caso de un hombre de 50 años, blanco, ingresado en el Servicio de Medicina Interna del Hospital Clínico Quirúrgico Docente "Faustino Pérez Hernández" por síndrome febril agudo, cefalea holocraneana, epistaxis y hemolacria. En el examen físico realizado se notó la salida de lágrimas con sangre, por el ángulo interno de ambos ojos y epistaxis. La inyección intradérmica en la cara dorsal del muslo izquierdo de 0,1 mL de sangre autóloga, no indujo reacción equimótica. La inyección de 0,1 mL de solución salina al 0,9 % como control en el muslo contralateral resultó negativa. Sobre la base del examen clínico y otras pruebas, se concluyó como un Síndrome de Gardner-Diamond. Esta infrecuente enfermedad debe ser considerada en el diagnóstico diferencial de un síndrome purpúrico de etiología no bien precisada, fundamentalmente en pacientes con problemas psiquiátricos (AU).

ABSTRACT The Gardner-Diamond syndrome, also known as psychogenetic purpura or erythrocyte autosensitization syndrome is very few frequent. The case of a white patient aged 50 years is presented. He entered the Service of Internal Medicine of the Teaching Clinic-surgical Hospital "Faustino Pérez Hernández" because of an acute fever syndrome, holocraneal headache, epistaxis and haemolacria. At the physical examination it was stated the flow of tears with blood, through the internal angle of both eyes and epistaxis. The intradermal injection of 0.1 ml of autologous blood in the left thigh dorsal side did not induce an ecchymotic reaction. The injection of 0.1 ml of 0.9 % saline solution as control in the contralateral side was negative. On the basis of the clinical examination and other tests, the authors arrived to the conclusion it is a Gardner-Diamond syndrome. This infrequent disease should be considered in the differential diagnosis of a purpuric syndrome of non-good précised etiology, mainly in patients with psychiatric problems (AU).

Síndrome de Gardner-Diamond. Presentación de un caso / Gardner-Diamond syndrome: A case report

RESUMEN El sindrome de Gardner- Diamond conocido también como púrpura psicógena o síndrome de autosensibilización eritrocitaria es muy poco frecuente. Se presenta el caso de un hombre de 50 años, blanco, ingresado en el Servicio de Medicina Interna del Hospital Clínico Quirúrgico Docente "Faustino Pérez Hernández" por síndrome febril agudo, cefalea holocraneana, epistaxis y hemolacria. En el examen físico realizado se notó la salida de lágrimas con sangre, por el ángulo interno de ambos ojos y epistaxis. La inyección intradérmica en la cara dorsal del muslo izquierdo de 0,1 mL de sangre autóloga, no indujo reacción equimótica. La inyección de 0,1 mL de solución salina al 0,9 % como control en el muslo contralateral resultó negativa. Sobre la base del examen clínico y otras pruebas, se concluyó como un Síndrome de Gardner-Diamond. Esta infrecuente enfermedad debe ser considerada en el diagnóstico diferencial de un síndrome purpúrico de etiología no bien precisada, fundamentalmente en pacientes con problemas psiquiátricos.

ABSTRACT The Gardner-Diamond syndrome, also known as psychogenetic purpura or erythrocyte autosensitization syndrome is very few frequent. The case of a white patient aged 50 years is presented. He entered the Service of Internal Medicine of the Teaching Clinic-surgical Hospital "Faustino Pérez Hernández" because of an acute fever syndrome, holocraneal headache, epistaxis and haemolacria. At the physical examination it was stated the flow of tears with blood, through the internal angle of both eyes and epistaxis. The intradermal injection of 0.1 ml of autologous blood in the left thigh dorsal side did not induce an ecchymotic reaction. The injection of 0.1 ml of 0.9 % saline solution as control in the contralateral side was negative. On the basis of the clinical examination and other tests, the authors arrived to the conclusion it is a Gardner-Diamond syndrome. This infrequent disease should be considered in the differential diagnosis of a purpuric syndrome of non-good précised etiology, mainly in patients with psychiatric problems.

Gardner-Diamond Syndrome: A Psychodermatological Condition in the Setting of Immunodeficiency.

Gardner-Diamond syndrome (GDS) is a psychological and dermatologic syndrome involving painful, ecchymotic, purpuric lesions that typically appear after a period of stress or minor trauma. This syndrome most commonly occurs in young women, though it has also been less commonly reported in men and adolescents. It is an uncommon condition and appropriate diagnosis is important to properly manage symptoms and minimize risks to patients. Here report a case of GDS presenting in a woman with common variable immunodeficiency (CVID), which, to the best of our knowledge, is not a comorbid condition that has previously been reported in correlation with GDS.

[Gardner-Diamond syndrome in a young man: A case report and literature review]. / Syndrome de Gardner-Diamond : à propos d'un cas chez un homme jeune et revue de la littérature.

INTRODUCTION: Gardner-Diamond syndrome is a rare condition secondary to a sensitization to self-erythrocytes. It is predominantly seen in women and presents as a painful ecchymotic disorder. An underlying psychiatric disease or a triggering psychological stress is of important diagnostic value. CASE REPORT: We report a 24-year-old patient who presented with intermittent spontaneous painful ecchymosis since 5 years. Complementary investigations failed to identify an organic disorder. Gardner-Diamond syndrome was retained because of the clinical presentation, the negativity of diagnostic work-up and the identification of a psychological trauma. Patient management (pain, psychological support) is difficult, justifying a multidisciplinary approach. CONCLUSION: Gardner-Diamond syndrome is a rare and unrecognized disorder, which should be discussed in the presence of ecchymotic or purpuric lesions that do not have a diagnostic orientation. Early recognition of this disorder enables initiation of an appropriate management, but also limits unnecessary additional explorations.

Gardner – Diamond Syndrome

Gardner–Diamond syndrome (GDS), or autoerythrocyte sensitisation, is a rare cause of recurrentpainful bruising with a typical anamnesis and prodrome. We describe a patient with GDS and discussthe literature surrounding this unique condition.

Síndrome de Gardner-Diamond como diagnóstico diferencial de lupus / Gardner-Diamond syndrome as a differential diagnosis of lupus

El síndrome de Gardner-Diamond o púrpura psicógena es una vasculopatía de presunto origen autoinmune que se caracteriza por una reacción cutánea localizada, asociada a situaciones de estrés emocional. Se presenta el caso de una paciente con lesiones equimóticas, dolorosas y de aparición intermitente, relacionadas con diversos eventos estresores, que habían sido manejadas como manifestación de lupus

Gardner-Diamond syndrome or psychogenic purpura is an vasculopathy characterized by a localized cutaneous reaction, associated with episodes of emotional stress or mental illness as trigger factors. A case of a female patient with multiple, intermittent, nodular, ecchymotic and painful lesions related to various stressing events that was treated as lupus is reported below

A Long-Term Psychological Observation in an Adolescent Affected with Gardner Diamond Syndrome.

Gardner-Diamond syndrome (GDS) is an uncommon disease clinically characterized by a wide spectrum of psycho-emotive symptoms associated with painful ecchymoses/purpuric lesions and positivity of auto-erythrocyte sensitization skin test. Herein, a perspective clinical and psychological observation of an adolescent GDS is firstly reported focusing on her psychological features long-term monitored for a 1-year period. The administration of a standardized tools battery allowed us to define psychological features of the young patient over time and to monitored clinical course and response to treatment.

A disease difficult to diagnose: Gardner-Diamond syndrome accompanied by platelet dysfunction.

Gardner Diamond syndrome is a rare condition characterized with painful ecchymoses in different parts of the body and cutaneous and mucosal hemorrhages. The etiology is not known fully and psychogenic factors are thought to be involved. Cutaneous lesions and hemorrhages develop mostly following emotional stress and rarely minor traumas and may recur. Although the extremities are involved with the highest rate, the lesions may be observed in any part of the body. Hemostatic tests are generally normal. The majority of the subjects is composed of young women. It is observed more rarely in men and children. In this article, a patient who presented with recurring painful echymoses and bleeding disorder and diagnosed with Gardner Diamond syndrome by intracutaneous injection of autologous blood was presented to emphasize that this syndrome is observed rarely in the childhood and should be considered not only in the differential diagnosis of cutaneous lesions, but also in the differential diagnosis of various system hemorrhages.

Perplexing purpura in two females: Rare case of autoerythrocyte sensitization syndrome.

Autoerythrocyte sensitization syndrome is a psychologically induced painful bruising condition. Two female, 19 and 30-year-old presented with recurrent episodes of painful ecchymotic bruising over accessible areas of body. In the younger female, episodes were since 3 years and were precipitated by stress and trivial trauma. The elder female presented with similar lesions since 3 months which were spontaneous in presentation. There were no obvious psychiatric manifestations in either. Clinically, ecchymotic changes in various stages of development were seen. Routine hemogram and coagulation profile were normal. Histopathology showed extravasated erythrocytes, perivascular neutrophils and fibrinoid deposition. Intradermal injection of autologous whole blood produced a painful ecchymotic reaction after 2 h similar to the presenting lesions. Psychiatric evaluation revealed mild mixed depression - anxiety disorder in the younger female while the latter revealed no abnormalities. The diagnosis of autoerythrocyte sensitization syndrome was made based on clinical history and findings, positive autoerythrocyte sensitization test, psychiatric evaluation and absence of any other clinical or laboratory pathology.

[Stigmata: From Saint-Francis of Assisi to idiopathic haematidrosis]. / Les stigmates: de Saint-François d'Assise à l'hématidrose idiopathique.

"Religious" stigmata describe body marks and sores that reflect the crucifixion wounds of Christ (hands and wrists, feet, flank, back, shoulders, forehead) and which are transferred to an individual through fervent prayer. "Non-religious" stigmata usually describe spontaneous episodes of bleeding with no identifiable cause outside any religious context. Irrespective of the setting in which they occur, such lesions are extremely striking. This review intends to summarize what is currently known about stigmata in the light of current medical knowledge, while stressing that the "spontaneous" nature of these lesions does not rule out a genuine medical cause.

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.

Gardner-Diamond syndrome associated with complex regional pain syndrome.

BACKGROUND: Gardner-Diamond syndrome (GDS) is also known as psychogenic purpura, autoerythrocyte sensitization syndrome and painful bruising syndrome. MAIN OBSERVATION: This is a case report of 27-year-old woman who presented with unexplained bruising and intramuscular hematomas after a seven year history of complex regional pain syndrome. Her evaluation consisted of hematological studies, skin and muscle biopsy; it failed to reveal an underlying coagulopathy, vasculitis or other demonstrable cause. In the absence of any other etiology, she was diagnosed as Gardner-Diamond syndrome. CONCLUSIONS: This patient is unique because of intramuscular hematomas and the association of Complex regional pain syndrome with Gardner-Diamond syndrome.

Antidepressant-induced Remission of Gardner Diamond Syndrome.

We describe the clinical presentation of a 25-year-old female patient who presented in dermatology with recurrent episodes of painful ecchymotic bruising over the anterior aspect of both arms and face. On enquiry, these episodes were precipitated by emotional stress and were preceded with a history of fall from the stairs. The patient also had multiple stressors in her day-to-day life and symptoms of depression. A diagnosis of mild depressive disorder without somatic complaints and Gardner Diamond syndrome was made. The patient was started on antidepressants, which not only improved her mood symptoms but also caused a remission of her painful bruises.

Síndrome de Gardner-Diamond.: Presentación de un caso / Gardner-Diamond syndrome:: A case presentation

Se presenta el caso de una mujer de 44 años, blanca, ingresada en el Servicio de Medicina Interna del Hospital Clinicoquirúrgico Docente "Miguel Enríquez" por síndrome febril agudo y hematuria. En el examen físico realizado se notó la salida de lágrimas con sangre muy fluida, por el ángulo externo del ojo izquierdo. La inyección intradérmica en la cara dorsal del muslo derecho de 0,1 mL de sangre autóloga, indujo una reacción equimótica dolorosa en el sitio de la inyección. La inyección de 0,1 mL de solución salina al 0,9 % como control en el muslo contralateral, resultó negativa. Sobre la base del examen clínico y estas pruebas, se concluyó como un Síndrome de Gardner-Diamond. Esta entidad debe ser considerada en el diagnóstico diferencial de un síndrome purpúrico de etiología no bien precisada, fundamentalmente en pacientes con problemas psiquiátricos.

This is the case of a white woman aged 44, admitted in the Internal Medicine Service of "Miguel Enríquez" Clinical Surgical Hospital due to acute febrile syndrome and hematuria. At physical examination performed we noted a very high level of watering with presence of blood by left angle of left eye. The intradermal injection of 0,1 ml of autologous blood in dorsal part of right thigh, induced a painful ecchymotic reaction in injection site. The injection of 0.1 ml of 0,9% saline solution as control in contralateral thigh was negative. On the base of clinical examination and these tests, we conclude that it was a Gardner-Diamond syndrome. This entity must to be considered in differential diagnosis of a purpuric syndrome of a non-well established etiology, mainly in patients presenting with psychiatric disorders.