Mirmohammad Sadeghi Hassan, Gholami Toghchi Sanaz, Mashhadiabbas Fatemeh, Baseri Milad, Mohammadalizadeh Chafjiri Maryam, Nikraftar Parnian, Khorsand Ardeshir
Department of Oral and Maxillofacial Surgery, Dentistry Faculty, Shahid Beheshti University of Medical Sciences, Velenjak St., Shahid Chamran Highway, Tehran, 1985717443, Iran.
Department of Oral and Maxillofacial Pathology, Dentistry Faculty, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
J Med Case Rep. 2025 Apr 3;19(1):155. doi: 10.1186/s13256-025-05062-z.
Langerhans cell histiocytosis is a rare disease of the reticuloendothelial system. This report presents a novel case of Langerhans cell histiocytosis with systemic involvement that started with a simple chief complaint. The reporting of this case raises awareness of the distinctive characteristics of this challenging disorder.
A 24-year-old male patient of Persian ethnicity presented with a chief complaint of halitosis following coronavirus disease 2019 recovery to his general dentist's office. Intraoral and extraoral examinations revealed no specific problem. In the follow-up session after phase I periodontal treatment, teeth sensitivity to cold stimulus was evident, and radiographs revealed a large lytic intraosseous lesion in the mandible. An incisional biopsy revealed Langerhans cells and a positive reaction to Langerin and cluster of differentiation 1a, thus, he was diagnosed with Langerhans cell histiocytosis. After performing positron emission tomography with fluoro-2-deoxyglucose and computed tomography, magnetic resonance imaging, and cone beam computed tomography, owing to generalized disease involvement, the patient was referred to an oncologist. Ultimately, it was found that the patient's childhood health issues, including endocrine problems, were likely caused by an undiagnosed Langerhans cell histiocytosis. The oncologist chose denosumab, vinblastine, etoposide, 6-mercaptopurine, methotrexate, and pegfilgrastim regimen. The follow-up was not possible as the patient died following an accident.
This reveals the vitality of the early diagnosis of Langerhans cell histiocytosis to prevent disease progression. Awareness of diverse and nonpathognomonic manifestations of Langerhans cell histiocytosis, proper medical interview and history taking, and the use of routine radiographs may aid clinicians in lowering morbidity and mortality rates associated with such conditions.
朗格汉斯细胞组织细胞增多症是一种罕见的网状内皮系统疾病。本报告介绍了一例以简单主诉起病、累及全身的朗格汉斯细胞组织细胞增多症新病例。该病例报告提高了人们对这种具有挑战性疾病独特特征的认识。
一名24岁的波斯族男性患者因2019冠状病毒病康复后出现口臭,前往其普通牙医诊所就诊。口腔内和口腔外检查未发现具体问题。在第一阶段牙周治疗后的随访中,牙齿对冷刺激敏感明显,X线片显示下颌骨有一个大的溶骨性骨内病变。切开活检显示朗格汉斯细胞,对 langerin和分化抗原簇1a呈阳性反应,因此,他被诊断为朗格汉斯细胞组织细胞增多症。在进行氟代脱氧葡萄糖正电子发射断层扫描以及计算机断层扫描、磁共振成像和锥形束计算机断层扫描后,由于疾病广泛累及,该患者被转诊至肿瘤学家处。最终发现,该患者童年时期的健康问题,包括内分泌问题,可能是由未确诊的朗格汉斯细胞组织细胞增多症引起的。肿瘤学家选择了地诺单抗、长春碱、依托泊苷、6-巯基嘌呤、甲氨蝶呤和聚乙二醇化重组人粒细胞刺激因子方案。由于患者在一次事故后死亡,无法进行随访。
这揭示了朗格汉斯细胞组织细胞增多症早期诊断对于预防疾病进展的重要性。了解朗格汉斯细胞组织细胞增多症的各种非特异性表现、进行适当的医学问诊和病史采集以及使用常规X线片,可能有助于临床医生降低与此类疾病相关的发病率和死亡率。