Os estigmas são marcas manifestadas fisicamente mas que tradicionalmente são reputadas como tendo origem espiritual, e que alguns acreditam reproduzirem as cinco chagas de Jesus Cristo. Os estigmas podem tomar formas variadas, como úlceras, chagas, ferimentos, queimaduras, bolhas e lacerações, dentre outros, e normalmente são verificados em um dos cinco pontos do corpo pelos quais Jesus teria sido pregados à cruz: pés, punhos e tórax. Enquanto festividade católica, em Portugal é realizada especialmente no dia 7 de fevereiro.
Introdução
O que você precisa saber de cara
Síndrome rara caracterizada por episódios de dor intensa e sangramentos espontâneos (articular, gastrointestinal, epistaxe, etc.), frequentemente associados a febre e lesões cutâneas. Pode haver histórico de comportamento autolesivo e anormalidades nos membros.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 22 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 42 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome de sensibilização auto-eritrocitária
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Case Report: Psychogenic purpura in a uremic patient on peritoneal dialysis.
Psychogenic purpura (Gardner-Diamond syndrome) is a rare autoimmune vasculopathy characterized by the spontaneous onset of painful edema and infiltrative cutaneous lesions that rapidly develop into ecchymosis after severe psychological stress events. In this article, we report an 87-year-old female uremic patient who was admitted to the hospital with erythema and subcutaneous ecchymoses on the head and face following an Aedes mosquito sting. She was previously diagnosed with "toxic insect stings and skin bacterial infections" and was given anti-infective treatment by an outside hospital, which was ineffective. Subsequent laboratory tests at our hospital revealed only an increase in fibrinogen and leukocytosis. Tracing the history revealed that the patient's purpura episodes were related to a major life event, the death of her husband. After consultation with the dermatology department, the patient's autoerythrocyte sensitization test was positive, and she was finally diagnosed with "psychogenic purpura". Treatment included glucocorticoids and immunomodulators, supplemented by anti-infective and renal replacement therapy, and the patient's ecchymosis gradually subsided and resolved after one month of follow-up. This case highlights the complexity of diagnosing psychogenic purpura and the significance of medical history in the diagnosis. Only accurate and timely diagnosis can effectively avoid unnecessary treatment.
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.
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.
Reprint of: Adolescent with leg rash.
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.
Publicações recentes
Case Report: Psychogenic purpura in a uremic patient on peritoneal dialysis.
Diagnosing the Dermatologic Blues: Systematic Review of the Rare Conundrum, Psychogenic Purpura.
Role of Autoerythrocyte Sensitization Test in the Diagnosis of Recurrent Spontaneous Bruising.
Reprint of: Adolescent with leg rash.
Is Gardner-Diamond syndrome related to autoimmunity?
📚 EuropePMC36 artigos no totalmostrando 9
Case Report: Psychogenic purpura in a uremic patient on peritoneal dialysis.
Frontiers in immunologyDiagnosing the Dermatologic Blues: Systematic Review of the Rare Conundrum, Psychogenic Purpura.
JMIR dermatologyRole of Autoerythrocyte Sensitization Test in the Diagnosis of Recurrent Spontaneous Bruising.
Indian dermatology online journalReprint of: Adolescent with leg rash.
Disease-a-month : DMIs Gardner-Diamond syndrome related to autoimmunity?
Northern clinics of IstanbulSpontaneous extracranial hemorrhagic phenomena, autoerythrocyte sensitization syndrome and anticardiolipin antibodies: Is there any relationship?
Indian journal of dermatology, venereology and leprologyClinical Spectrum of Autoerythrocyte Sensitization Syndrome: A Series of Five Cases.
Indian dermatology online journal[Gardner-Diamond syndrome in a young man: A case report and literature review].
La Revue de medecine internePsychogenic Purpura (Gardner-Diamond Syndrome).
The primary care companion for CNS disordersAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Case Report: Psychogenic purpura in a uremic patient on peritoneal dialysis.
- Diagnosing the Dermatologic Blues: Systematic Review of the Rare Conundrum, Psychogenic Purpura.
- Role of Autoerythrocyte Sensitization Test in the Diagnosis of Recurrent Spontaneous Bruising.
- Reprint of: Adolescent with leg rash.
- Is Gardner-Diamond syndrome related to autoimmunity?
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:324636(Orphanet)
- MONDO:0017943(MONDO)
- GARD:6481(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q7124887(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
