Multisystemic presentation of langerhans cell histiocytosis in pediatric patient: a case report of letterer-siwe disease

IF 1.9 3区医学 Q2 DENTISTRY, ORAL SURGERY & MEDICINE

Oral Surgery Oral Medicine Oral Pathology Oral Radiology Pub Date : 2025-03-14 DOI:10.1016/j.oooo.2025.01.008

Larissa Santos Amaral Rolim, Helder Domiciano Dantas Martins, Eduarda Gomes Onofre de Araújo, Keila Martha Amorim Barroso, Ana Carolina Lyra de Albuquerque, Alexandre Rolim da Paz, Paulo Rogério Ferreti Bonan

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引用次数: 0

Abstract

Letterer-Siwe disease is the most common and serious of these entities, affecting mainly infants up to 2 years of age. Here we report a case affecting a 1-year-old white male, presented with a complaint of nonhealing gingival lesion. Medical history revealed a supposed milk protein allergy, gastric reflux, anorexia, recent fever, and seborrheic dermatitis. On extraoral physical examination, palpable bilateral anterior and postauricular cervical lymph nodes were noted, along with abdominal distension, fever, and seborrheic dermatitis. Intraoral examination revealed bilateral gingival enlargement in the posterior tooth region. Subsequently, an incisional biopsy of the gingival lesion revealed ulcerative areas with intense mixed inflammatory infiltrate. Immunohistochemical evaluation confirmed positivity for CD1a, CD68, S100, Myeloperoxidase, CD20, and CD3, confirming the diagnosis of Langerhans cell histiocytosis. Due to multisystemic presentation, the diagnosis of Letterer-Siwe disease was confirmed, and the patient was referred for chemotherapeutic treatment under follow-up.

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小儿朗格汉斯细胞组织细胞增多症的多系统表现：letter -siwe病1例报告

letter - siwe病是这些疾病中最常见和最严重的，主要影响2岁以下的婴儿。在这里，我们报告一个病例影响1岁的白人男性，提出了一个投诉，不愈合的牙龈病变。病史显示疑似牛奶蛋白过敏、胃反流、厌食症、近期发烧和脂溢性皮炎。口外体格检查，可触及双侧颈前及耳后淋巴结，伴有腹胀、发热和脂溢性皮炎。口腔内检查发现双侧后牙区牙龈肿大。随后，牙龈病变的切口活检显示溃疡区伴有强烈的混合炎症浸润。免疫组化检查证实CD1a、CD68、S100、髓过氧化物酶、CD20、CD3阳性，诊断为朗格汉斯细胞组织细胞增多症。由于多系统表现，确诊为letter - siwe病，并在随访中转介化疗。

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来源期刊

Oral Surgery Oral Medicine Oral Pathology Oral Radiology DENTISTRY, ORAL SURGERY & MEDICINE-

CiteScore

3.80

自引率

6.90%

发文量

1217

审稿时长

2-4 weeks

期刊介绍： Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology is required reading for anyone in the fields of oral surgery, oral medicine, oral pathology, oral radiology or advanced general practice dentistry. It is the only major dental journal that provides a practical and complete overview of the medical and surgical techniques of dental practice in four areas. Topics covered include such current issues as dental implants, treatment of HIV-infected patients, and evaluation and treatment of TMJ disorders. The official publication for nine societies, the Journal is recommended for initial purchase in the Brandon Hill study, Selected List of Books and Journals for the Small Medical Library.