Abstract：
BACKGROUND: Primary Immune Thrombocytopenic Purpura (ITP) is an autoimmune hematological condition characterized by isolated thrombocytopenia and frequently presents with oral manifestations. However, reports of primary ITP in patients with Turner Syndrome (TS) are exceptionally rare, with few cases documented in the literature. Herein, we describe an unusual case of primary ITP with exuberant oral manifestations in a patient with TS.
METHODS: A 29-year-old woman was referred to an oral diagnostic service with complaints of \"blood blisters and gum bleeding\" lasting 8 h. On extraoral physical examination, multiple petechiae were observed in the upper and lower limbs, in addition to hemorrhagic extravasation in the right ocular sclera (hyposphagma). On intraoral examination, multiple vesicles and blisters filled with blood were identified on the lower lip, back of the tongue, and buccal mucosa, along with spontaneous gingival bleeding and hemorrhagic petechiae on the palate. Laboratory tests revealed thrombocytopenia (5000/mm3), whereas the blood count showed normality in the red and white series. After excluding other etiological factors or associated diseases, the patient was diagnosed with severe ITP and began treatment with systemic corticosteroids in the intensive care unit, resulting in a successful increase in platelets. After a 2-year follow-up, the patient remains free of ITP recurrences.
CONCLUSIONS: Oral manifestations may be one of the first signs of ITP. Therefore, it is essential that dentists are familiar with the condition and, when faced with unusual oral bleeding, consider the possibility of a hematological disorder such as ITP, ensuring a correct and early diagnosis. Moreover, the presence of ITP can further exacerbate complications associated with TS. Therefore, rigorous follow-up of these patients is crucial, considering the high incidence of cardiovascular and autoimmune diseases and the reduced life expectancy of these patients.
METHODS: A 29-year-old woman was referred to an oral diagnostic service with complaints of \"blood blisters and gum bleeding\" lasting 8 h. On extraoral physical examination, multiple petechiae were observed in the upper and lower limbs, in addition to hemorrhagic extravasation in the right ocular sclera (hyposphagma). On intraoral examination, multiple vesicles and blisters filled with blood were identified on the lower lip, back of the tongue, and buccal mucosa, along with spontaneous gingival bleeding and hemorrhagic petechiae on the palate. Laboratory tests revealed thrombocytopenia (5000/mm3), whereas the blood count showed normality in the red and white series. After excluding other etiological factors or associated diseases, the patient was diagnosed with severe ITP and began treatment with systemic corticosteroids in the intensive care unit, resulting in a successful increase in platelets. After a 2-year follow-up, the patient remains free of ITP recurrences.
CONCLUSIONS: Oral manifestations may be one of the first signs of ITP. Therefore, it is essential that dentists are familiar with the condition and, when faced with unusual oral bleeding, consider the possibility of a hematological disorder such as ITP, ensuring a correct and early diagnosis. Moreover, the presence of ITP can further exacerbate complications associated with TS. Therefore, rigorous follow-up of these patients is crucial, considering the high incidence of cardiovascular and autoimmune diseases and the reduced life expectancy of these patients.
摘要：
背景：原发性免疫性血小板减少性紫癜（ITP）是一种自身免疫性血液学疾病，其特征是孤立性血小板减少症，通常表现为口腔表现。然而,特纳综合征（TS）患者的原发性ITP报告非常罕见，文献中记录的案例很少。在这里，我们描述了一例不寻常的原发性ITP患者,其口腔表现旺盛.
方法：一名29岁的妇女被转诊到口腔诊断服务，主诉“血泡和牙龈出血”持续8小时。在上肢和下肢观察到多个瘀点，除了右眼巩膜的出血性外渗（hyposphma）。在口腔检查中，在下唇发现了多个充满血液的囊泡和水泡，舌头的背面，和颊粘膜，伴有自发性牙龈出血和上颚出血性瘀斑。实验室检查显示血小板减少症（5000/mm3），而血液计数在红色和白色系列中显示正常。排除其他病因或相关疾病后，该患者被诊断为严重的ITP，并在重症监护病房开始使用全身性皮质类固醇治疗，导致血小板成功增加。经过2年的随访,患者仍无ITP复发.
结论：口腔表现可能是ITP的首发症状之一。因此,牙医必须熟悉这种情况，当面临不寻常的口腔出血时，考虑血液病的可能性，如ITP，确保正确和早期诊断。此外，ITP的存在可进一步加剧与TS相关的并发症.因此,对这些患者进行严格的随访至关重要，考虑到心血管和自身免疫性疾病的高发病率以及这些患者的预期寿命缩短。
方法：一名29岁的妇女被转诊到口腔诊断服务，主诉“血泡和牙龈出血”持续8小时。在上肢和下肢观察到多个瘀点，除了右眼巩膜的出血性外渗（hyposphma）。在口腔检查中，在下唇发现了多个充满血液的囊泡和水泡，舌头的背面，和颊粘膜，伴有自发性牙龈出血和上颚出血性瘀斑。实验室检查显示血小板减少症（5000/mm3），而血液计数在红色和白色系列中显示正常。排除其他病因或相关疾病后，该患者被诊断为严重的ITP，并在重症监护病房开始使用全身性皮质类固醇治疗，导致血小板成功增加。经过2年的随访,患者仍无ITP复发.
结论：口腔表现可能是ITP的首发症状之一。因此,牙医必须熟悉这种情况，当面临不寻常的口腔出血时，考虑血液病的可能性，如ITP，确保正确和早期诊断。此外，ITP的存在可进一步加剧与TS相关的并发症.因此,对这些患者进行严格的随访至关重要，考虑到心血管和自身免疫性疾病的高发病率以及这些患者的预期寿命缩短。
