Palatal Petechiae in the Absence of Group A Streptococcus in Pediatric Patients with Acute-Onset Neuropsychiatric Deterioration: A Cohort Study.

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 with hematuria and sexual pain disorder: a case report]. / Hematüri ve Cinsel Agri Bozuklugunun Eslik Ettigi Psikojenik Purpura; Bir Olgu Sunumu.

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.

A case of psychogenic purpura in a female child.

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.

Autoerythrocyte sensitization syndrome with vertigo and hemilateral auditory impairment.

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.

Undiagnosed purpura: a case of autoerythrocyte sensitization syndrome associated with dermatitis artefacta and pseudo-ainhum.

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.

Bathtub suction-induced purpura.

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.

Periodic painful purpura: fact or factitious?

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.

Autoerythrocyte sensitization (Gardner-Diamond) syndrome mimicking compartment syndrome.

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.