Spontaneous Oral Purpura in Immune Thrombocytopenia
Jakob L. Drivenes, Anette Bygum
N Engl J Med 2024; 390:1029
DOI: 10.1056/NEJMicm2312260
A 76-year-old man presented to the dermatology clinic with a 2-day history of blood-filled blisters on the tongue. The lesions had appeared suddenly without antecedent trauma. He reported no other bleeding symptoms. The physical examination was notable for multiple hemorrhagic bullae on the tongue and gingiva, and purpura were noted on the arms and legs. The platelet count was 3000 per cubic millimeter (reference value, 150,000 to 400,000). The results of other laboratory studies, including tests for human immunodeficiency virus, cytomegalovirus, Epstein–Barr virus, severe acute respiratory syndrome coronavirus 2, and hepatitis B and C, were normal. A peripheral-blood smear showed thrombocytopenia with megakaryocytes. Bone marrow biopsy showed only an elevated number of megakaryocytes. A diagnosis of immune thrombocytopenia was made. Primary immune thrombocytopenia is a diagnosis of exclusion that is made after other causes of thrombocytopenia and secondary immune thrombocytopenia are ruled out. In rare cases, atraumatic development of purpura on the oral mucosa in the absence of other bleeding symptoms may be the first manifestation of the condition. Treatment with an 8-week tapering course of prednisolone was given. At the 12-week follow-up, the patient’s mucocutaneous lesions had abated, and the platelet count had normalized.
