Autoerythrocyte Sensitization Research Articles

Four cases, one syndrome: Gardner-Diamond syndrome

Four cases, one syndrome: Gardner-Diamond syndrome

Psychogenic Ecchymosis along with Positive Autosensitization to Own Red Blood Cells: A Clue to Gardner Diamond Syndrome

Psychogenic Ecchymosis along with Positive Autosensitization to Own Red Blood Cells: A Clue to Gardner Diamond Syndrome

A Gardner-Diamond Syndrome Accompanied by Complex Regional Pain Syndrome.

A Gardner-Diamond Syndrome Accompanied by Complex Regional Pain Syndrome.

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

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. 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. 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. 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. 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.

Case Report of Gardner-Diamond Syndrome

Gardner-Diamond syndrome or psychogenic purpura is a rare vasculopathy disorder characterized by spontaneous painful purpuric rash without a history of trauma or antiplatelet or anticoagulant therapy. It occurs in women with mental health disorders. Gardner and Diamond in 1955 described a syndrome of auto erythrocyte sensitization which presented in abnormal crops of painful inflammatory ecchymosis in 4 patients at the site of trauma followed by progressive erythema and edema. We present a 52-year-old age woman who was admitted to the internal medicine department and referred for dermatological consultation for recurrent spontaneous purpuric eruptions. The patient was under treatment for depression for many months. Her investigations and coagulation profile were nearly within normal.

Role of Autoerythrocyte Sensitization Test in the Diagnosis of Recurrent Spontaneous Bruising.

Psychogenic purpura, also known as autoerythrocyte sensitization syndrome, is a rare condition which is characterized by spontaneous development of painful purpura, or ecchymoses. Skin lesions are usually preceded by stress and emotional trauma. It is usually a diagnosis of exclusion after ruling out history of trauma, drug intake and other bleeding disorders. Autoerythrocyte sensitization test (AEST) and a psychiatric evaluation helps in the diagnosis and treatment. To demonstrate the importance of AEST in diagnosing the patient of Gardner Diamond syndrome. Five suspected cases of autoerythrocyte sensitization syndrome underwent AEST after ruling out other causes of bleeding. Four out of five patients were positive for AEST while one patient was negative. Psychiatric complaints were present in three patients. One patient was lost to follow up. Rest all patients responded well to vitamin C supplementation. Autoerythrocyte sensitization syndrome is a rare disorder and is a diagnosis of exclusion, so a thorough workup of the patient to rule out common causes of bruising is essential. A high index of suspicion on the clinician's part and a simple OPD-based AEST may help in the diagnosis. Psychiatric consultation is important to find out the stress factor and timely management.

A psychopathological reading of the blood sweat phenomenon and religious stigmas: The case of Blessed Elena Aiello.

Around 300 cases of stigma have been described since the 13th century. Hematidrosis, hemolacria, bloody otorrhea, vicarious menstruation, and Gardner-Diamond syndrome are dermatological manifestations that are also observed in cases of stigma. This paper presents the disconcerting circumstances in the life of the Blessed Elena Aiello. From 1923 until her death, she suffered severe hematidrosis blood sweating on her forehead, hands, feet and knees, just as stigmata appear at the crucifixion of Jesus Christ. Stigmas, in Christian tradition, are marks corresponding to those left on Christ's body by the torture and the Crucifixion, it's said that are impressed on the person body by divine favor. These episodes, among the few recognised as authentic by the Catholic Church would appear each Friday in Lent, particularly on Good Friday. In this unique case study, doctors Fabrizio and Turano performed a medical study where the origin of the sores was unclear. Neurologist and psychiatrist Vincenzo Bianchi, on assessing the case, raised the possibility of simulation or mere suggestion on the part of the patient. Keywords: Elena Aiello, Autosuggestion, Psychology, Stigmata, Psychopathology, Malingering, Factitious Disorder.

Somatization in dermatology.

Medically unexplained dermatologic symptoms, such as pruritus, numbness and burning are known as somatization. These cutaneous symptoms can be very difficult to treat because of an absence of an objective explanation and they may not fit neatly into any known dermatological or psychiatric condition. These disorders are more commonly encountered in primary care and in dermatology, rather than in psychiatry. Certain skin disorders, for example, pruritus, could be a manifestation of somatization and others may predispose to somatic symptoms, for example, atopic dermatitis and psoriasis. Although there has been increasing research in the interconnection between psychiatry and dermatology, psychodermatology is a relatively new crossover discipline in clinical practice and recognition of psychodermatological conditions, such as cutaneous somatic disorders, can be difficult. Somatization may occur with or without the existence of a dermatological disease. When a dermatological disorder is present, somatization should be considered when the patient is worrying too much about their skin, spending too much time and energy on it and especially if the patient also complains of many non-cutaneous symptoms. Purely cutaneous somatic conditions include for example, the genital pain syndromes or Gardner-Diamond syndrome, characterized by unexplained bruising, which usually affects women. Effective management tools may include mindfulness therapies, pharmacotherapy with selective serotonin reuptake inhibitors, tricyclic antidepressants and cognitive conduct therapy. Electroconvulsive therapy can also be considered in extremely rare cases for treatment of severe somatization on a background of mood disorders. This paper discusses somatization, its relationship to immunodermatoses and its relevance to clinical practice.

AUTOERYTHROCYTE SENSITIZATION SYNDROME.

Autoerythrocyte sensitization syndrome is characterized by lesions which are ill dened, tender, recurrent and spontaneous in nature . There are fewer than 200 case reports[1]. A 40 year old married female, agricultural labourer by occupation came to our OPD with history of recurrent painful petechiae and ecchymosis over both upper and lower extremities since 7 years. The episodes were frequent and were heralded by pain and edema of legs, followed by petechiae progressing to ecchymosis within a day. Lesions occurred at intervals of 7 to 10 days. Not associated with itching ,fever, joint pains or any other symptoms. She was excessively worried since her mother's demise and had low level of mood when visited. It typically occurs in middle-aged females with underlying psychiatric disorders such as depression, anxiety or obsessive compulsive disorders.

Painful blue finger: Achenbach's syndrome.

A previously healthy 60-year-old male patient presented with sudden pain and bruising in his left little finger, which occurred at home shortly before his visit. At the time, he noticed a bluish discolouration on the palmar aspect of the finger and became concerned that it might be a sign of a serious vascular or haematological disease. He had no history of preceding trauma, cold exposure or medication. Physical examination revealed a subcutaneous haematoma with slight swelling but without coldness or paresthesia (figure 1A). Allen's test and capillary refilling were normal. Laboratory tests revealed a normal platelet count and coagulation function. Based on these findings, Achenbach's syndrome (paroxysmal finger haematoma) was clinically diagnosed, and the patient was observed without treatment. The discolouration and pain resolved spontaneously 6 days later (figure 1B). Achenbach's syndrome was first described in 1958 by Walter Achenbach.1 It is a rare cause of finger pain characterised by ecchymotic discolouration with sudden pain onset in the fingers or hands. Although the aetiology is unknown, it is a self-limiting condition found predominantly in middle-aged women.2 The diagnosis is based on the typical clinical presentation, and examinations reveal no specific abnormalities. The differential diagnosis includes acute limb ischaemia, trauma, Raynaud's syndrome, acrocyanosis, Gardner-Diamond syndrome and chilblain.2 The most emergent differential diagnosis is acute limb ischaemia, which can be distinguished from Achenbach's syndrome by the non-involvement of the digit tips.3 Clinicians should be aware of this entity to avoid unnecessary tests and to reassure patients of its benign prognosis.

Management of Psychiatric Disorders with HIV and Dermatological Disorders.

Management of Psychiatric Disorders with HIV and Dermatological Disorders.

GARDNER DIAMOND SYNDROME: A CASE REPORT

Gardner Diamond Syndrome (GDS) is a rare autoimmune disorder also known as autoerythrocyte sensitization syndrome represented with skin lesions. These lesions mostly occur after a triggering factor. The pathophysiology of the disease is not completely understood yet. In this case report, the characteristic features of GDS is presented; furthermore, our aim is to emphasize the effect of emotional stress during the disease.

Psychogenic purpura – remembering a rare image

Psychogenic purpura – remembering a rare image

Psychiatric Profile and Response to Combined Pharmacologic and Psychotherapeutic Treatment in Psychogenic Purpura.

Objective: Psychogenic purpura is a rare psychocutaneous entity characterized by painful atraumatic, ecchymotic, and purpuric lesions that commonly occur in young women. The objective of this study is to describe the clinical presentation, psychiatric profile, and response to therapy (pharmacologic and psychotherapeutic) in patients with psychogenic purpura.Methods: Twenty-three patients, referred from various clinical departments with unexplained purpura or ecchymotic lesions, were assessed in the department of psychiatry of a tertiary care institute in eastern India over a period of 3 years (May 1, 2017, to April 30, 2020). Relevant systemic evaluation and routine biochemical and laboratory tests including complete blood count, bleeding time, clotting time, prothrombin time, coagulation profile, and platelet function test were conducted for all patients to rule out an underlying organic cause. Associated psychiatric comorbidities were diagnosed as per the International Classification of Diseases, 10th edition. All patients who were eventually diagnosed with psychogenic purpura received appropriate treatment (pharmacologic and psychotherapy) and were followed up for 1 year.Results: Seven female patients (age range, 14-70 years, mean age = 30.28 ± 17.42 years) were diagnosed with psychogenic purpura. All patients had significant stressors prior to the appearance of lesions. Facial involvement was reported in 2 patients (28.6%). Multiple sites were affected in 4 patients (57.1%). The underlying psychiatric conditions diagnosed were depressive disorder (42.9%), generalized anxiety disorder (28.6%), dissociative convulsion disorder (14.3%), and panic disorder (14.3%). Associated personality disorder was noted in 2 patients (28.6%). All patients were started on selective serotonin reuptake inhibitors and concurrent psychotherapy. A short course of benzodiazepine was used in all patients and was subsequently tapered off. At 1-year follow-up, 2 patients reported recurrences, while others continued to do well with no similar episodes.Conclusions: Appropriate psychiatric evaluation and necessary intervention in patients with psychogenic purpura lead to improved patient outcomes.

The Mayo Clinic Experience With Psychogenic Purpura (Gardner-Diamond Syndrome)

BackgroundThe objective of this study was to describe presentation, natural history, management and long-term outcomes of patients with psychogenic purpura (PP), also known as Gardner-Diamond Syndrome. MethodsIn this retrospective study, records of patients with a diagnosis of PP seen at Mayo Clinic, Rochester from 1976 to 2016 were reviewed. Available literature regarding PP was also comparatively reviewed. ResultsSeventy-six patients with a diagnosis of PP were identified and 54/76 (71%) experienced a prodromal sensation. The Condensed MCMDM-1 bleeding score, excluding cutaneous manifestations, was <3 in 91% of patients. Laboratory tests of primary and secondary hemostasis were normal. Fifty-four percent of patients had an underlying psychiatric diagnosis. Management approaches included psychological counseling and psychiatry evaluation in 44 patients. Pharmacologic treatment for 30 patients included psychotropic agents, antihistamines, hormonal medications and anti-inflammatory agents. At a median follow-up of 5years (range 1-34),13/28 (46.4%) experienced recurrent ecchymoses and 6 continued to seek hematology follow-up at Mayo Clinic, Rochester. Our data was similar to the aggregate data from case reports in the literature. ConclusionsFor patients with unexplained recurrent ecchymosis a diagnosis of PP should be considered. Diagnosis is one of exclusion and initial evaluation should include documenting a bleeding score and obtaining laboratory tests assessing primary and secondary hemostasis. The relatively low bleeding scores together with laboratory assessments support that PP is primarily a dermal rather than a systemic bleeding diathesis. In our cohort, addressing psychological stressors was the most effective treatment; however pharmacologic therapy can be used for refractory disease.

Report of two cases with autoerythrocyte sensitization syndrome

Auto-erythrocyte Sensitization Syndrome, also known as Gardner Diamond syndrome, is characterized by spontaneous painful and repetitive purpura. Here, we report two women aged 14 and 18 years who were diagnosed with auto-erythrocyte sensitization syndrome by typical laboratory, clinical, histopathological features and intradermal skin test. Burning and itching were the major complaints associated with spontaneous ecchymosis. The frequency of attacks in the patient using a specific serotonin re-uptake inhibitor due to migraine was significantly higher than the patient who did not receive psychiatric treatment. It was concluded that careful psychiatric evaluation is important for effective treatment of the disease.

Gardner‐Diamond syndrome: a systematic review of treatment options for a rare psychodermatological disorder

Gardner-Diamond syndrome (GDS) is a rare psychodermatological condition characterized by the formation of spontaneous, painful skin lesions that develop into ecchymosis following episodes of severe physiological or psychological stress. The majority of GDS cases occur in young adult females, and although the etiology of this rare disorder is unknown, there appears to be a psychological component correlated with the coexistence of previous psychiatric diagnoses. Due to the rare nature of this disorder, there exist few guidelines for prompt clinical diagnosis and optimal treatment. Here, a systematic review was conducted to include 45 cases of patients with GDS to better understand clinical presentation as well as current treatment options. Ultimately, GDS is a diagnosis of exclusion after other coagulopathies and causes of purpura are ruled out. High clinical suspicion following laboratory and clinical exclusion of known physiological causes is necessary for diagnosis. Selective serotonin reuptake inhibitors (SSRIs) and corticosteroids are cost effective first line treatments for GDS with proven efficacy in symptomatic relief. GDS refractory to initial treatment may require regular psychotherapy and titrated SSRI dosages to achieve long-term success. This review of available case studies serves to comprehensively describe the clinical presentation and available treatment approaches to this rare psychodermatological disorder.

Religious stigmata: a dermato‐psychiatric approach and differential diagnosis

Stigma refers to the wounds reproduced on the human body, similar to the ones inflicted on the Christ during his crucifixion, on the palms, soles, and head, as well as the right or the left side of the chest, the lips and, the back. Whether they are genuine or fabricated, stigmata are still considered a medical enigma. To summarize and analyze all available medical data about stigmata, integrating both its dermatological and psychiatric features. A systematic search of Pubmed and Embase databases was conducted on August 19, 2016, using the terms stigma, stigmata, and religious. Around 300 cases of stigma have been described since the 13th century. Hematidrosis, hemolacria, bloody otorrhea, vicarious menstruation, and Gardner-Diamond syndrome were all dermatological disorders that resulted in signs and symptoms similar to that of stigma. Stigma was often associated with the occurrence of intense emotion or great psychological stress. It also occurred in the context of simulation, factitious disorder, conversion disorder, and functional neurological symptom disorder. Stigma remains an example of the intricate relationships existing between medicine, psychiatry, psychology, spirituality, and the human body.

Painful bruising syndrome: A case report

Painful bruising syndrome: A case report

Syndrome de Gardner-Diamond : à propos d’un cas chez un homme jeune et revue de la littérature

IntroductionGardner–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 reportWe 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. ConclusionGardner–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.