Medical History

Hypertension.

Medications

Hydrochlorothiazide 25 mg.

Review of Systems

All systems within normal limits.

• Vital Signs
  • Blood pressure: 126/88 mmHg
  • Respiration: 16 breaths/min
  • Pulse: 80 beats/min

Social History

Denies.

Dental History

Regular dental recall.

Restorative treatment.

Maxillary third molars (#1 and #16) extracted 22 years prior.

Extraction of teeth #17, #18, and #19 two weeks prior due to severe mobility.

Head and Neck Examination

Intraoral Exam

Soft Tissue

Gingival tissues of teeth #s 2–6, 20, and #s 29–32 demonstrate marked erythema and enlargement.

Extraction sockets of teeth #s 17–19 demonstrate marked erythema and redundant soft tissue.

Generalized gingival recession.

All other oral mucosal surfaces appear healthy with no visible signs of inflammation, infection, neoplasia, or other pathology.

Hard Tissue

See Figure 1.6.1 panoramic radiograph and Figure 1.6.2 Periodontal Chart.

Minimal plaque and calculus accumulation.

Radiographic Findings and Problem List

• Well‐circumscribed radiolucency in the left posterior mandible extending from the ascending ramus to tooth #20 and involving the inferior alveolar nerve canal.
• Well‐circumscribed radiolucency in the right posterior mandible extending from the apices of tooth #32 to the interproximal bone between teeth #28 and #29.

Clinical Impressions

1. Multiple, large bony defects of this type can often be identified in certain populations including adults with advanced chronic severe periodontitis, young adults with a history of aggressive periodontitis, and patients with syndromes predisposing them to rapid periodontal bone such as Down or Papillon‐Lefevre syndrome. This pattern of bone loss, in a patient of this age and health status is uncommon. Furthermore, the loss of attachment pattern and the patient age of do not fit well for either variant of aggressive periodontitis or chronic periodontitis. The differential diagnosis would include both infectious and neoplastic processes. The initial examination should focus on identifying any source of pulpal necrosis of the involved teeth that could be responsible for the defect, such as a fractured tooth or gross carious lesion. Once a pulpal etiology has been ruled out, the remaining entities on the differential diagnosis may include aggressive periodontitis, a benign neoplastic process such as ameloblastoma, and a malignant neoplastic process such as lymphoma. A biopsy is appropriate to arrive at a definitive diagnosis in this clinical scenario.

Treatment Plan

• Referral to oral and maxillofacial surgery for an incisional biopsy of the tissue involved with the radiolucencies.

Discussion

The gingiva, periodontal ligament, cementum, and alveolar bone are the four components that comprise the periodontium. These components work together providing structural support to the dentition. Assessment of periodontal health depends on the periodontal exam. This exam includes four parts: the medical history, the dental history, radiographic exam, and clinical exam (Newman et al. 2012). The medical history may reveal critical information related to periodontal health. For instance, DM has been strongly correlated with periodontal health in the scientific literature (Chapple et al. 2013). Identification of a history of DM is likely to impact the periodontal status of the patient. The dental history is important for determining the patient’s past and ongoing treatment, current oral hygiene regimen, and general attitude toward oral health (Newman et al. 2012). Following the medical and dental history, a radiographic exam is usually required. For details concerning the radiographic exam, please see Case 3 of this chapter, Radiographic Examination.

With the medical and dental history obtained and radiographic studies completed, the clinical examination of the periodontium can commence. This exam consists of several steps. As emphasized thus far in the text, deciding on a systematic, repeatable method for performing this task is foundational. Critical elements of any methodology should contain at least the following steps: assessment of mobility, sensitivity to percussion, plaque, and calculus accumulation, a gingival examination, inspection of all dental restorations, assessment of periodontal pocketing and furcation involvement, and detailed notation of all findings. Please see Table 1.6.1 for a suggested methodology.

Periodontal attachment loss is most commonly related to chronic periodontitis. The American Academy of Periodontology (AAP) classifies slight to moderate loss of periodontal support as periodontal pockets up to 6 mm and clinical attachment loss of up to 4 mm. Advanced loss of periodontal support is classified as pocketing greater 6 mm and clinical attachment loss greater than 4 mm (American Academy of Periodontology 2000a). While loss of periodontal support is most often mediated by chronic periodontitis, aggressive periodontitis (AP), although less common, can result in similar clinical outcomes. AP progresses more rapidly than the chronic variant and generally occurs in patients who are healthy and have a level of plaque and calculus that do not seem to correspond to the level of attachment loss. Two variants of AP exist; a localized variant, commonly occurring around adolescence, and a generalized variant with a predilection for those under 30 years of age. Localized AP typically affects the permanent incisors and first molars; generalized AP involves three permanent teeth besides the incisors and first molars (American Academy of Periodontology 2000b).

A cursory review of the panoramic radiograph and periodontal charting of the patient in this case may appear as some form of severe periodontitis. However, a closer study of this patient reveals a pattern that does not correspond to either chronic or aggressive periodontitis. The severe loss of attachment is isolated to the posterior lower right and left quadrants. The loss of attachment would be more generalized to fit a diagnosis of generalized AP or chronic periodontitis. A diagnosis of localized AP is questionable as the central incisors are not involved and the patient is outside the age group where this disease is typically identified. It is critical to consider other disease processes when a clinical pattern does not fit into the diagnostic parameters of the common variants of periodontitis. These considerations should prompt the clinician to refer the patient to an oral and maxillofacial surgeon for an incisional biopsy. The definitive diagnosis in this case was LCH.

LCH is characterized by a destructive proliferation of histiocyte‐like cells (Badalian‐Very et al. 2011; Neville et al. 2015). The first designation given to this disease was “histiocytosis X.” The term used more commonly today, LCH, points to the phenotypic characteristics shared by the disease and the Langerhans cells of epidermal and mucosal surfaces. In the past it was believed that disease cells originated from the Langerhans cells. Recent research indicates that this may not be the case with the most likely origin of the cells being a myeloid‐derived precursor (Badalian‐Very et al. 2011).

The presentation of this disease may take several forms. The disease may be solitary, or multifocal involving various anatomic sites including skin, mucosa, bone, lymph nodes, soft tissue, and organs. In the past the terms Hand‐Schuller‐Christian disease and Letterer‐Siwe disease were used to describe chronic and acute disseminated LCH, respectively. These eponyms have largely been set aside and replaced with a description of the focus of disease and sites of involvement (Neville et al. 2015; Badalian‐Very et al. 2011).

LCH is diagnosed in patients over a wide age range. However, over 50% of cases are diagnosed in those younger than 15 years. The disease demonstrates an equal distribution between the sexes (Neville et al. 2015).

LCH is capable of destroying hard tissue and results in radiolucent lesions on radiography. Destruction of tooth‐supporting bone can produce a similar radiographic pattern to that seen in severe periodontitis (Neville et al. 2015). It is important to keep LCH on one’s differential diagnosis in cases of atypical periodontal bone loss, particularly in pediatric patients. Lesions involving bone may present with symptoms of tenderness or dull pain (Neville et al. 2015). When lesions are diagnosed in the oral soft tissues they often present initially as an ulcerative mass (Neville et al. 2015).

Diagnosis of LCH is based on histopathological review of biopsied specimens coupled with immunohistochemical evaluation (Neville et al. 2015). Treatment depends upon the extent of disease involvement. Mainstays of therapy have included curettage, local steroid injection and radiation for isolated, single focus disease (Neville et al. 2015; Badalian‐Very et al. 2011). Disseminated disease is often treated by a combination of systemic steroids and chemotherapeutics (Neville et al. 2015; Badalian‐Very et al. 2011). The discovery of the BRAF^V600E mutation in 38–69% of LCH cases has opened the possibility of molecular‐targeted therapy (Badalian‐Very et al. 2011; Haroche et al. 2013). The prognosis varies from patient to patient and depends upon several factors including age at the time of diagnosis, the degree of dissemination as well as the specific organs involved in the disease process (Neville et al. 2015). Some cases of LCH have exhibited spontaneous resolution, while others will lead to the patient’s demise.

It is critical that oral health‐care providers become familiar with the presenting signs of LCH as the oral and maxillofacial region is not an uncommon site of involvement. Identification of the disease and a timely referral is likely to have a positive impact on the patient’s long‐term prognosis.