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Background: Palatal petechiae are 95% specific for streptococcal pharyngitis. Despite this, and despite prior research demonstrating that Group A Streptococcus (GAS) is a common antecedent to pediatric acute-onset neuropsychiatric syndrome (PANS) episodes, we anecdotally observed a low rate of documented GAS in patients with PANS and palatal petechiae. This retrospective chart review was conducted to formally report the rate of palatal petechiae and concurrent GAS in a cohort of patients with PANS and investigate other etiologic factors., Methods: The clinical notes of 112 patients seen at the Stanford PANS Clinic who met PANS research criteria were reviewed for mention of palatal petechiae. The medical records of patients who demonstrated palatal petechiae on physical examination were reviewed for signs of infection, a clinical history of trauma, and laboratory results that could indicate other causes of petechiae., Results: Twenty-three patients had documented palatal petechiae on physical examination (ages 5-16, 13/23 [57%] male). Fifteen patients had a rapid GAS test and GAS culture in the Stanford PANS clinic, all with negative results. Evidence of recent GAS infection was found in 8/23 (32%) patients (elevated GAS titers [n = 6] or documentation of a positive rapid GAS test at another facility [n = 2]), one of whom also had potential herpes simplex virus (HSV) infection. One patient had potential HSV infection and recent palatal trauma. No patients had thrombocytopenia. 14/23 (61%) of patients with palatal petechiae had no discernable cause of petechiae. 10/19 (53%) of patients had antihistone antibodies., Conclusions: Despite the established relationship between palatal petechiae and GAS, no patient with palatal petechiae in our clinic tested positive for GAS and only 32% had evidence of recent GAS. Most did not have an identifiable cause for the palatal lesions. This finding suggests the potential for alternative causes of palatal petechiae or undetectable GAS in our patient population. The high prevalence of palatal petechiae without GAS infection suggests that the pathogenesis of PANS is multifactorial and may involve disruption or inflammation of the microvasculature. Additional research is needed to further elucidate these findings.

Psychogenic purpura (Gardner-Diamond syndrome) is the occurrence and spontaneous recurrence of painful ecchymosis following emotional stress and minor trauma. Although the exact mechanism of this syndrome remains unknown, apart from skin lesions, different types of hemorrhaging have been reported, such as epistaxis, gastrointestinal bleeding, and bleeding from the ear canals and eyes. We report a psychogenic purpura case that presented with hematuria in addition to skin lesions. Based on the psychiatric evaluation she was diagnosed with major depressive disorder, generalized anxiety disorder, and obsessive-compulsive disorder. Additionally, sexual pain disorder accompanied these disorders. With the help of antidepressant and supportive psychotherapy, the patient's ecchymosis and bleeding disappeared. During 8 months of follow-up the symptoms did not return. Vaginismus has not been reported in patients with psychogenic purpura. The presence of vaginismus, which is seen more frequently in eastern cultures and is thought to be related to sociocultural determinants, suggests that some cultural factors may be common to both psychogenic purpura and vaginismus. The aim of this case report was to call attention to a syndrome that is rarely seen and diagnosed, and to discuss its relationship to psychosocial factors. This syndrome should be considered in the differential diagnosis of not only ecchymotic lesions, but also various types of bleeding, including hematuria. Despite the fact that its etiology and treatment are not clearly understood, it should be noted that psychological factors play a role in this disease and therefore, psychopharmacological and psychotherapeutic approaches can be effective.

Autoerythrocyte sensitization syndrome (AES) is characterized by recurrent, painful purpura or ecchymosis. Testing for the reappearance of lesions after injection of the patient's own erythrocytes is usually useful for the diagnosis of AES, but the significance of this test is still controversial. As the lesions often appear in patients with psychiatric disorders, mental factors such as depression and stress are considered to be involved in the occurrence and exacerbation of AES. We report a 28-year-old woman who presented recurrent episodes of painful purpura with vertigo and hemilateral auditory impairment after difficulties at her workplace. After the diagnosis of AES, she was referred for psychiatric counselling, after which the symptoms disappeared. These findings suggest that treatment for psychological disorders is important in patients with AES.

Recurrent spontaneous cutaneous bruising or bleeding through intact skin may be due to underlying emotional stress which may be due to auto-erythrocyte sensitisation. A 9-year-old female child presented with recurrent episodes of oozing from the skin of both lower eyelids and lower peri-orbital areas along with bleeding from tongue which ceased spontaneously after a few minutes without having any induration or oedema at the sites. The child had no systemic abormality, but she was nervous and attention seeking. Psychologic assessment revealed that she was suffering from conversion anxiety and somatoform disorder. After twelve psychotherapeutic sessions she showed marked improvement and her symptoms did not recur.

A 23-year-old young woman presented with recurrent episodes of painful bruising along with linear erosions on the accessible areas of the body of nine years duration with a pseudo-ainhum of her left nipple for the past three months. Her case history included repeated visits to various physicians at different centers and an extensive investigative profile. A diagnosis of autoerythrocyte sensitization was made on the basis of the clinical history, dermatological examination complemented by a positive autoerythrocyte sensitization test, psychiatric evaluation and absence of any organic cause for her ailment. She was placed on psychiatric management and has remained symptom-free after six months follow-up. The case is reported for its rarity, as well as for the association of autoerythrocyte sensitization syndrome with frank dermatitis artefacta and pseudo-ainhum, which to the best of our knowledge has not yet been reported in the literature.

We report two cases of bathtub suction-induced purpura. Two girls, ages 9 and 14 years, developed recurring purpura "spontaneously" in the same lower back location and in the same U-shaped distribution. The patients had similar histopathologic findings, with extravasated erythrocytes and a sparse superficial inflammatory infiltrate. After extensive evaluation, one patient eventually admitted to self-inducing her lesions by creating suction against a bathtub. The second patient denied self-inflicting her lesions; however, her purpura improved when she stopped taking baths.

A 60-year-old woman with multiple psychosocial issues presented with a history of spontaneous painful bruising on her left upper arm. Extensive investigations did not reveal any abnormality apart from an elevated activated partial thromboplastin time as a result of factor XII deficiency. An autoerythrocyte sensitization test reproduced the tender bruises on her back. Our patient reported relief of pain with promethazine injections. She was subsequently referred to the psychiatric team for ongoing assessment and management. Having an awareness of this rare condition will assist in the prevention of unnecessary investigation of such cases and will allow the early referral of patients for appropriate psychological counselling.

We report a case with an unusual manifestation of autoerythrocyte sensitization syndrome (Gardner-Diamond syndrome). The patient had the clinical signs of compartment syndrome of the forearm requiring fasciotomy. 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.

Psychogenic purpura, also known as recurrent painful bruising or autoerythrocyte sensitization syndrome (Gardner-Diamond syndrome) is usually associated with emotionally disturbed patients. It is a troublesome entity for both patient and physician since extensive work-ups yield no diagnosis. We describe two females in their early twenties with recurrent painful bruising and diverse accompanying symptoms which appeared after physical trauma. One of the patients developed a bruise after intradermal injection of her own blood (with no reaction to saline injection). One patient had a personality (borderline) disorder, the other a factitious disorder. Punch biopsies revealed a perivascular inflammatory infiltrate, erythrocyte extravasation and no vasculitis. Psychogenic hemorrhagic disorders are uncommon yet must be considered in the differential diagnosis of purpura. Patients are usually young emotionally troubled females who present painful recurrent bruises on extremities frequently after trauma or surgery. Autoimmune mechanisms and increased cutaneous fibrinolytic activity have been implicated, although further studies are needed. Correct diagnosis is important to avoid aggressive and even mutilating treatments, and an adequate comprehension of these purpuras is important for the attending physician.

Recurrent episodes of a "purple chin" in an adolescent girl eluded definitive diagnosis for three years. The condition occurred premenstrually, but results of extensive tests failed to reveal any abnormal hormonal sensitivity. A diagnosis of factitial purpura was eventually established.

Stress is a term that is readily recognized by everyone but defines rigorous scientific definition. It is widely interpreted as the emotional and biologic responses to novel or threatening situations. In humans, however, the term "distress" seems to be preferable, more clearly defining the fact that is the response that is being referred to, rather than the stimulus. Distress has been postulated to be capable of precipitating an overt illness, as when it occurs coincidentally with an incipient infection or neoplasm. Moreover, it is able to provoke several disorders and symptoms in many tissues and organs, including the skin. In this paper the authors focus on the main characteristics of stress and emphasize the relevance of psychosomatic medicine that proposes the simultaneous examination of psyche and soma. Special consideration is given to a peculiar form of skin disease, psychogenic purpura, together with the stigmata of mystics that, in large part, seem to be conditioned or provoked (or provocable) by emotional stress or by psychic influences on cutaneous fibrinolytic activity.

A case of autoerythrocyte sensitization syndrome is reported. This syndrome most often appears in young women who have an underlying emotional disorder; features include bizarre, tender ecchymotic lesions, which are most commonly located on the arms and legs. Systemic symptoms often accompany the onset of these lesions. A diagnosis of autoerythrocyte sensitization syndrome may be made in a patient who has the typical history and clinical picture of the syndrome and in whom a skin test with use of the patient's blood reveals a positive reaction.

Psychogenic "purpura" consists in the spontaneous appearance of recurrent bruising, it is a reality which is still unexplained. Most often woman with an underlying emotional disorder are affected. In addition to cutaneous ecchymoses and hematomas they may have menometrorrhagia, hematuria, epistaxis and gastrointestinal bleeding in addition to many other complaints encompassing multiple organ systems. Cutaneous bruising is heralded by a burning or stinging sensation followed after a few hours by local warmth, puffiness and erythema, most often with some itching. The pain subsides when the ecchymoses appear a day later or earlier. Blood coagulation and hemostatic tests remain normal in all patients. A rare but poorly studied variety of psychogenic bleeding are religious or other stigmata with periodic bleeding at hands and feet, under the left breast and at the forehead, occasionally with bloody tears. It would be risky to substitute one poorly understood diagnosis (stigmata) with another (hysterical conversion). It is very difficult to distinguish spontaneous psychogenic from selfinflicted iatrogenic bleeding (purpura factitia) because patients may have the same obsessive-compulsive behavior or hysterical trait. Moreover, patients with self-induced bleeding act in a most clever manner and their continuous observation is difficult. The differential diagnosis with the "battered child" syndrome is rather easy. Other bleeding disorders with normal coagulation tests are an inherited failure of the collagen biosynthesis (f.i. Ehlers-Danlos syndrome) or congenital or acquired vascular disorders (purpura senilis of Bateman, vitamin C deficiency, morbus Schamberg, purpura annularis of Majocchi, orthostatic purpura). There is virtually no scientific information on the interaction between the nervous system and hemostasis or blood coagulation. The only therapeutic approach in patients with psychogenic bleeding is psychiatric with particular attention to the sociocultural background of the patient and his family.

Three identical case histories consisting of circular delimited petechial purpura around the mouth and chin in children aged 13-15 years are presented. This form of purpura was undoubtedly due to self-mutilation by establishing of a vacuum over the skin produced by a tumbler from which the air had been partially aspirated. This is regarded as a symptom of hysterical conversion on account of stressing conditions at home or in school. Strategies for making the patient and the parents aware of the causal connection and the possibilities for intervention are mentioned.

This paper presents a 26-year-old woman with the characteristic features of Gardner-Diamond syndrome: recurrent painful bruises reproducible by intradermal injection of autologous red blood cells, and a "hysterical" personality pattern together with nonspecific multisystem complaints.

A case of psychogenic purpura is likely to be unforgettable for everyone concerned. The patient's bizarre and dramatic appearance can frighten both the patient and medical personnel unfamiliar with the condition, causing panic and intense demands for drastic, inappropriate remedies. Our second experience with this condition was such a memorable case. We stress how difficult it can be to withstand the pressures of colleagues, the patient, and his or her family, and how important it sometimes is to be resolute regarding the need for conservative management.

We have investigated a female patient with autoerythrocyte sensitization syndrome (AES syndrome), having a positive skin response to her own red blood cells (RBC) and to phosphatidylserine (PS). Using 2,4,6-trinitrobenzenesulfonic acid (TNBS), bee venom phospholipase A2 and merocyanine 540 binding, we have demonstrated that in RBC of patient more than 50% of PS is redistributed into the outer leaflet of the plasma membrane. Using homologous RBC from a healthy donor we were able to induce transbilayer PS redistribution by incubation with the patient plasma. The presence of immunoglobulin E against cardiolipin and PS was proved in patient's plasma. We elaborated a method for cytoskeleton visualization using indirect immunofluorescence technique. We found disorders in cytoskeleton organization in RBC of the patient. We recommend in vitro testing for AES syndrome diagnosis. The positive effect of chlorpromazine treatment is described.

Autoerythrocyte sensitization syndrome is characterized by sudden and spontaneous painful bruising and swelling occurring in young female patients with psychiatric disorders. This syndrome may present with such severe swelling of involved areas that aspiration or excision may be needed to relieve pressure. The presentation of this condition in the emergency department must be differentiated from more common coagulopathies. Differentiation from the self-mutilation disorder, which often presents to the ED, can be difficult. Although the pathogenesis is uncertain, purpuric lesions are reproduced by injections of washed red cell erythrocyte stroma, suggesting an autoimmune etiology. We describe a patient with recurrent bruising diagnosed in the ED who responded with classic inflammation to autologous injection of red blood cells.

A case of Gardner-Diamond's syndrome is reported in which the lesions were localized to the face. The syndrome is characterized by the spontaneous onset of recurrent painful ecchymoses mainly in young women. The elective site of the lesions is on the limbs, sometimes on the abdomen or thorax, and only rarely on the face. They are accompanied by a very rich symptomatology contrasting with normal biological test results. An autoerythrocytic sensitization mechanism has been suggested but few biological arguments exist to support this auto-immune hypothesis of its origin. In contrast, the psychological background is hysterical in type, and the affection can be included among the psychogenic purpuras.

The important psychological functions of the human skin - the skin functions as a border, contact and sense organ and as an organ of expression - as well as causalgenetic aspects of psychodermatology and the most important psychodermatological diseases are reported. Frequent psychosomatic dermatoses like chronic urticaria (bibliography), pruritus (case report, perioral dermatitis and alopecia areata (case reports) are described. In atopic dermatitis, psoriasis vulgaris and acne rosacea psychological factors are involved in addition to constitutional factors. Psychovegetative dermatoses can be due to situational problems (stress), but dermatologic symptoms may also have a mere appellant character (e.g. slight effluvium). Dermatologic symptoms may occur in connection with psychoses (e.g. parasitophobia). Psychic dermatologic symptoms may also arise secundary to severe chronic dermatoses. In psychoneurotic diseases such as dermatitis arterficialis skin is the target organ. Therapeutical aspects are considered.

Experience at University Hospitals of Cleveland with 71 cases of Gardner and Diamond's syndrome of autoerythrocyte sensitization is reviewed. Gardner and Diamond attributed the pathogenesis of the inflammatory bruises typical of this syndrome to sensitization to the stroma of the patients' own erythrocytes, as demonstrated by reproduction of the lesion on intracutaneous injection of erythrocytic stroma. Nearly all the cases my colleagues and I have seen were in adult women, in whom the onset of inflammatory bruising could often be precisely dated, frequently some weeks after an injury or surgical procedure or, more often, severe emotional stress. Bouts of bruising were often preceded by sensations localized to the affected site. Cutaneous responses to the injection of erythrocytes were erratic. The patients described a wide range of both hemorrhagic and nonhemorrhagic complaints, including, among others, severe headaches, paresthesias, repeated syncope, diplopia (sometimes monocular), and "nervousness." Psychiatric studies indicated that patients had overt depression, sexual problems, feelings of hostility, and obsessive-compulsive behavior. The patients had traits that can be described as typical of a hysterical character disorder. Therapy of autoerythrocyte sensitization--that is, psychogenic purpura--has been difficult; in younger individuals, psychiatric therapy has appeared to be beneficial.

We have seen six examples of autoerythrocyte sensitization in children that allow a definition of the disorder in the pediatric age group. The typical skin lesion is a painful, erythematous bruise that starts after minor trauma or surgery and often involves an area away from the injury site. The skin manifestations can be debilitating, and reappear unpredictably for an indefinite period. Somatic symptoms can be associated with the bruising. Usually the children have a disturbed psychological background. Characteristically, multiple detailed investigations are performed before the diagnosis is made. An intradermal injection of autologous red cells may or may not give a "positive" ecchymotic reaction. Pediatric patients seem to respond well to psychotherapy, which is usually the only effective form of treatment.

A 27 year old women revealed chronic relapsing, spontaneously developing hematomas without coagulopathy. The lesions were reproducible by intradermal injection of erythrocyte membranes as well as DNA. Positive test reactions could be elicited or suppressed by psychological influences on the patient. This case and similar reports in the literature raise the question whether Gardner-Diamond syndrome and the autosensitization to DNA are different entities.

The case deals with an 18-year-old female patient suffering from psychogenic purpura who was successfully treated with individual and family oriented dynamic psychotherapy. Emphasis is placed on family dynamics and their possible bearing on the origin of the illness.

Autoerythrocyte sensitization, a disease characterized by recurrent and painful ecchymoses, was renamed psychogenic purpura by Agle and Ratnoff in 1968. The patients, usually female, share certain described personality characteristics. The case is reported of a 20-year-old woman who had suffered from the disease since age 12. She was treated for 3 years in intensive psychotherapy resulting in a complete remission of her symptoms of bleeding.

The occurrence of psychogenic purpura has been documented in adults. The authors present their experience with nine adolescent and young adult patients observed since 1981. The age of eight of these patients ranged from 13 to 18 years. One patient with cystic fibrosis was 23-years-old. All patients presented with painful inflammatory lesions followed by bruising. There was no evidence of an infectious process or a hematologic, immunologic, or renal abnormality. Integral to the diagnosis was the existence of an underlying emotional disorder. In all patients, bruising decreased after diagnosis was explained to the patient and family. Four patients required psychotherapy. Results of 2-year follow-up suggest that early intervention may produce a favorable outcome in this age group.