Primary immune thrombocytopenic purpura with exuberant oral manifestations in a patient with Turner syndrome

• Published in: Special care in dentistry Vol. 44; no. 6; pp. 1564 - 1571
• Main Authors: Santos, Liz Oliveira Trajano dos, Santos, Angélica de Sousa, Oliveira, Marielly de Souza, Lopes, Natalia Vitória de Araújo, Oliveira Júnior, José Klidenberg, Medeiros, Hianne Cristinne de Morais, Cunha, John Lennon Silva, Barnabé, Luan Éverton Galdino
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01-11-2024
• Subjects: Autoimmune diseases; Bleeding; Blood; Buccal mucosa; Cleft lip/palate; Corticosteroids; Extravasation; Genetic disorders; Hematological diseases; Hematology; Hemorrhage; immune thrombocytopenic purpura; Life span; Patients; Purpura; Thrombocytopenia; Thrombocytopenic purpura; Turner syndrome; Turner's syndrome; Turner syndrome; immune thrombocytopenic purpura; hemorrhage
• ISSN: 0275-1879; 1754-4505; 1754-4505
• DOI: 10.1111/scd.13039

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.
Case report
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.
Conclusion
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.