Langerhans' cell histiocytosis diagnosed through periodontal lesions: a case report

BACKGROUND: Langerhans' cell histiocytosis (LCH) is a rare disorder in which a neoplastic proliferation of cells similar to the Langerhans' cell of the epidermis is observed. The disease may affect different organs as solitary or multiple lesions with a complete dissemination in different organs such as the bone (jaws), lung, hypothalamus, skin and mucous membranes, lymph nodes, liver, and other tissues. Although LCH is considered a childhood or juvenile disease, the diagnosis is often made in adults, and in many cases, a juvenile form progresses into adult life. Hand-Schuller-Christian disease (HSCD) is an LCH form in which the typical lesions Involve the cranial bones, the eyes, and the pituitary gland. METHODS: This article describes a case of a patient diagnosed, through periodontal lesions and diabetes insipidus, as having Langerhans' cell histiocytosis. CONCLUSION: As far as LCH lesions appearing in periodontal tissues, the periodontist should be involved in the detection of oral lesions.     

Langerhans' cell histiocytosis - A Case Report

Langerhan's histiocytosis was formerly known as histiocytosis X and refers to a group of conditions characterized by the uncontrolled stimulation and proliferation of a normal antigen-processing cell, the Langerhan's cell. Mandibular involvement associated with LCH is uncommon in a young child. Most reports describing bone involvements in LCH have mainly referred to male subjects over 20 years of age. The purpose of this report is to describe a case of multifocal bony LCH with mandibular involvement in a 8 year old girl and to discuss the appropriate management of such a case.     

Langerhans' cell histiocytosis restricted to the oral mucosa

Langerhans' cell histiocytosis comprises a group of disorders with Langerhans' cell proliferation as a common feature. The clinical presentation might be highly varied. Typically, there is bone involvement and, less frequently, lesions might be found in other organs, particularly the lungs, liver, lymph nodes, skin, and mucosae. Lesions limited to the oral mucosa are rare. We describe two patients with unifocal eosinophilic granuloma exclusively limited to the oral mucosa. Triamcinolone acetonide infiltration of a palatal lesion yielded a good result in one patient. Six months later, a similar lesion developed in the mandibular gingival mucosa, but it responded to the same treatment. The lesion in the other patient responded to local radiotherapy.     

Oral mucosal involvement in Langerhans' cell histiocytosis: long-term follow-up of a rare case

Langerhans' cell histiocytosis (LCH) is a rare disease where different organs and systems may be affected. Oral involvement generally consists of mucosal ulceration associated with lesions of the underlying bone. Many reports exist about the misdiagnosis of this disease. Various symptoms may lead the clinician to an incorrect diagnosis, especially with multiple organ involvement. Oral manifestations are common, and dentists should be aware of this disease and evaluate intraoral findings accordingly. This study presents an LCH case characterized by oral mucosal ulcerations with no involvement of the underlying bone. A definitive diagnosis was made by open biopsy from the oral mucosa.     

Periodontal disease associated with Langerhans' cell histiocytosis: case report

A clinical case of Langerhans' cell histiocytosis, type eosinophilic granuloma, in a young adult patient is presented. Because of the occurrence of oral manifestations in initial stages of the disease, there is a need for a differential diagnosis, especially with the early-onset periodontitis.     

小儿下颌骨郎格汉斯组织细胞增生症1例报告

小儿下颌骨郎格汉斯组织细胞增生症在临床上非常少见,作者报道1例收治病例,并结合有关文献资料对其临床表现、影像学表现、病理诊断及鉴别诊断、治疗方案进行讨论。认为小儿下颌骨郎格汉斯组织细胞增生症少见,且临床表现不典型,也无特征性的影像学表现,确诊只能依靠病理检查。郎格汉斯细胞增生是其特征性镜下改变,S-100和CD1a的免疫组织化学检查有助于诊断和鉴别诊断,其治疗以尽早手术切除结合密切随访为主。     

Langerhans' cell histiocytosis in association with periapical granulomas and cysts.

This report describes a series of six cases of inflammatory periapical disease with small aggregates of Langerhans cells as a minor component. Immunohistochemical findings confirm that the cells are phenotypically related to Langerhans cells. Aggregates of these cells are not normally found in radicular cysts or periapical granulomas and have been interpreted to represent chronic localized Langerhans' cells histiocytosis. Whether these lesions, which arise within the context of chronic inflammatory periapical disease, represent incipient eosinophilic granulomas or are a more benign, minimally destructive form of Langerhans' cell histiocytosis is unknown. Clinical follow-up suggests that these lesions remain localized and that curettage is adequate treatment.     

Squamous cell carcinoma of the tongue in 5-year-old girl with dyskeratosis congenita

Dyskeratosis congenita is a rare inherited bone marrow failure syndrome with three distinct clinical features: nail dystrophy, reticular skin pigmentation, and oral leukoplakia. The case of a 5-year-old female patient diagnosed with squamous cell carcinoma of the tongue is reported here. An autosomal dominant type 3 TINF2 mutation subsequently confirmed the diagnosis of dyskeratosis congenita. The traditional tongue cancer treatment was adapted for this young patient. While the tongue cancer lesions and leukoplakia were removed, the deep margins were minimized to preserve the tongue muscles and flap surgery was avoided. Additional conservative measures were applied to suppress new leukoplakia lesions.     

Langerhans cell histiocytosis: recurrent lesions affecting mandible in a 10-year-old patient

Hand-Schuller-Christian disease is a multifocal variant of eosinophilic granuloma, characterised by the classical triad of bony lesions, exophthalmos and diabetes insipidus. This case relates recurrent Langerhans' cell histiocytosis lesions presented as destruction of periodontal support associated with diabetes in a 10-year-old patient. Medical history suggests that the case represents a case of Hand-Schuller Christian disease.     

Periodontal manifestation of Langerhans' cell histiocytosis in a young man: case report with a 24-month follow-up.

A 25-year-old male patient with skin lesions of Langerhans' cell histiocytosis (LCH) was referred for periodontal treatment because of bilateral severely inflamed ulcerative periodontal lesions at the mandibular second premolar and first molar. On the left side, significant bone loss was revealed. Anti-infectious and surgical periodontal therapy resulted in significant probing pocket depth reduction and clinical attachment gains that were maintained for up to 24 months after surgery. Biopsies were taken during periodontal surgery. Immunohistochemical staining revealed that the periodontal lesions were uncommon periodontal manifestations of LCH. Reports of oral manifestation of LCH and treatment methods are reviewed.     

Severe periodontitis in a 5-year-old girl with hyperimmunoglobulin E syndrome

The hyperimmunoglobulin E syndrome (HIES) is a multisystem disorder that affects the: (1) dentition; (2) skeleton; (3) connective tissues; and (4) immune system. Little is known about periodontal manifestations of the syndrome. The purpose of this report was to describe a 5-year-old girl with suspected autosomal-recessive HIES syndrome who revealed profusely bleeding and painful gingiva and generalized aggressive periodontitis. A polymerase chain reaction (PCR)-based microbiological examination detected Porphyromonas gingivalis, Tannerella forsythia, Prevotella nigrescens, Treponema denticola, Eikenella corrodens, and Campylobacter rectus in the deep periodontitis lesions. The extraction of all deciduous teeth due to a poor prognosis and risk of systemic infection led to resolution of the oral inflammation. Long-term follow-up is required to determine the periodontal prognosis of the erupting permanent teeth.     

Langerhans' cell histiocytosis of the jaw bones. Report of 11 cases

Eleven cases of Langerhans' cell histocytosis of the jaw bones are reported. The clinial and radiographical features are described, and the role of the dentist in the diagnosis and management of this disorder is discussed.     

Langerhans cell histiocytosis: oral/periodontal involvement in adult patients

Objective:  Langerhans cell histiocytosis (LCH) is a clonal proliferative multisystem disease. Although bone and mucosae have been classified as non-risk organs, their involvement may increase the risk of disease progression. Oral and periodontal lesions are burdened with a significant impairment of quality of life for associated signs, symptoms and loss of function. Most of information regards paediatric disease; the disease in adults has received limited attention.
Subjects and Methods:  A total of 31 adult patients affected by immuno-histopathology confirmed LCH have been prospectively examined; attention was paid to the occurrence and characterization of oral lesions.
Results:  Twelve patients developed oral lesions. Posterior regions of jawbones were always affected; the involvement of anterior regions was not constant. Unifocal oral involvement was significantly associated with multisystemic disease while multifocal lesions were associated with unisystemic disease. Oral disease presented with soft tissue ulcers (50% of cases), gingival bleeding (66.7%), pain (83.4%), periodontal damage (50%), tooth mobility (16.7%), non-healing extraction socket (8.3%); 41.6% of patients complained of negative outcomes on quality of life. Oral lesions were easily handled with local measures.
Conclusions:  Posterior regions require attention; single oral lesions may be part of multisystemic disease; oral and periodontal lesions may be early signs of disease reactivation.     

Langerhans' cell histiocytosis of the jaw bones. Report of 11 cases

Eleven cases of Langerhans' cell histocytosis of the jaw bones are reported. The clinial and radiographical features are described, and the role of the dentist in the diagnosis and management of this disorder is discussed.     

Mucoepidermoid carcinoma of the palate in a 5-year-old girl: case report and literature review

Background

Minor salivary gland tumors in children are uncommon. However, despite the low overall incidence, there is a high incidence of malignancy in these young patients which is reported to be above 50 %, with mucoepidermoid carcinoma (MEC) predominating. We hope that this case report will contribute to the enlightenment of the behavior and prognosis of pediatric MEC of palatal minor salivary gland tumors.

Case report

A 5-year-old girl was admitted to our center with a histologically confirmed MEC of the left hard palate. Primary incision for drainage of a supposed abscess and secondary biopsy had been performed elsewhere 14 days before. After further workup, we excised the tumor and the adjacent bone; clear margins of 10 mm were revealed. Adjuvant therapy was not necessary. The defect was allowed to undergo secondary wound healing. As of the 1-year follow-up, there had been no recurrences.

Conclusion

For differential diagnosis, MEC should be considered in cases of soft, slow-growing, painless, pale bluish-purple lumps of the palate even in young patients. Especially for nonresponders to initial treatment, early biopsy is recommended for histological confirmation or exclusion of a minor salivary gland tumor.     

Sturge-Weber syndrome in a 6-year-old girl

Sturge-Weber syndrome is a congenital disorder characterized by vascular facial birthmarks and neurological abnormalities. Oral cavity involvement may occur, and the extent of the vascular abnormality may vary considerably. The present authors report the case of a 6-year-old girl with Sturge-Weber syndrome, focusing on the clinical and radiographic features. Her dental management involved a multidisciplinary team and included orthodontic treatment using removable appliances.     

Primary squamous cell carcinoma of the maxillary alveolus in a 10-year-old girl

Oral squamous cell carcinoma is rare in the pediatric population. When it does occur, it is most commonly seen on the tongue or lip. In this article, we report a rare case of squamous cell carcinoma of the maxillary gingiva and alveolus in a 10-year-old girl and review the literature concerning oral squamous cell carcinoma in the pediatric population.