Description
● Case Summary
➣ Gender and Age: 3-year-old boy
➣ The main complaint is that the upper and lower jaws have had painful gingival swellings for a month.
➣ Background of Current Illness:
• No history of trauma, systemic illness, or comparable family history;
• Pain: abrupt onset, intermittent, non-radiating;
• Aggravated by mastication, alleviated on its own;
• Previous dental appointment one month prior—treated with analgesics and antibiotics, but symptoms remained.
● General Clinical Findings:
➣ The child is healthy and of a moderate build.
➣ Extraoral:
• Firm and sensitive, with a diffuse swelling (4 × 2 cm) over the left side of the jaw.
• Firm and non-tender lymph nodes in the left submandibular region can be felt (0.5 × 2 cm).
➣ Intraoral:
• Spotted bleeding across gingiva of 55, 65, 75, and 85; diffuse erythematous swellings with ulceration and necrotic slough.
• The impacted teeth have grade II mobility.
• Small bleeding patches on an ulcerated mucosa—a sign of soft tissue involvement.
● Investigation
➣ Haemoglobin:
• 10.5 g% (mild anaemia) is the result of haematological testing.
• Microcytic hypochromic pattern in peripheral smear.
• Normal CRP (2.5 mg/dl).
• Liver and renal function is normal.
HBsAg and HIV are negative.
➣ Radiological Findings:
• Intraoral radiograph: Mild radiolucency surrounding roots 55, 74, and 75.
• Orthopantomogram: The left mandible shows several regions of bone loss.
• 3D CT: Verified many osteolytic lesions involving the alveolar process, body, and ramus of mandible;
• CT (axial and coronal): many soft tissue density lesions with irregular "punched-out" bony destruction including left mandible, left occiput, right maxilla, and right temporal bone.
➣ Histology
• A hypercellular lesion with singly dispersed Langerhans cells with grooved (or "coffee-bean") nuclei and eosinophilic cytoplasm.
• Binucleated or multinucleated cells are present.
• The history of eosinophils, plasma cells, and lymphocytes.
• Langerhans Cell Histiocytosis (LCH) diagnostic results.
★ diagnosis
➣ Multifocal Langerhans Cell Histiocytosis (Eosinophilic Granuloma type) is the final diagnosis.
➣ The left mandible, right maxilla, right temporal bone, and left occipital bone are the sites that are affected.
●Discussion
➣Type of Disease: LCH is an uncommon condition that mostly affects children aged 1 to 15 years, with a higher prevalence in males. It is characterised by the clonal proliferation of antigen-presenting Langerhans cells.
➣ Pathophysiology: Monoclonal proliferation evidence makes Langerhans cells cancerous; abnormal Langerhans cell proliferation destroys soft tissue and bone.
➣Category:
• Acute disseminated form of Letterer-Siwe illness (infants).
• Chronic distributed form of Hand-Schüller-Christian illness (many bones + extraskeletal).
• Chronic localised eosinophilic granuloma: single or numerous bone lesions, without visceral involvement.
• This example is consistent with the type of eosinophilic granuloma.
Skull, mandible, maxilla, ribs, and vertebrae are common sites.
• Most often, the posterior mandible is impacted.
The following are examples of oral manifestations: gingival enlargement, bleeding, ulceration, discomfort, tooth movement, halitosis, and the appearance of "floating teeth."Oral lesions were the initial and sole manifestation in this instance.
Several "punched-out" osteolytic lesions without sclerotic boundaries are among the radiological features.
•In extreme situations, lesions combine, potentially leading to pathological fracture and cortical perforation.
Different diagnoses include multiple myeloma, cyclic neutropenia, metastatic cancer, Papillon-Lefevre syndrome, and chronic periodontitis.
Protocol
The course of treatment
➣Localized Lesions: Low-dose radiation, intralesional corticosteroids, or surgical curettage.
Systemic chemotherapy (vinblastine, corticosteroids, methotrexate, and cytarabine) for multisystem disease.
➣ Recurrence is decreased by vincristine and prednisone combination therapy.
Prognostic markers include age, number of lesions, and organ failure. ➣Prognosis: • Better in localised forms; bad in children under 2 years or with multisystem involvement.
● Interventions and Management
As shown below, the management strategy was determined by the location and severity of the lesions.
➣ Rapid Medical Assistance
• Due to multisystem bone involvement, local lesions were not surgically addressed at this stage; instead, the kid was transferred to a cancer treatment centre for additional systemic treatments.
➣ Overview of Therapeutic Intervention
• For localised lesions, surgical excision or curettage is recommended.
• Lesions that are less accessible or recurrent: Low-dose radiation therapy may be used sparingly.
• Intralesional corticosteroid therapy: Prednisolone (20–30 mg/day) is used for 2-4 weeks with tapering to effectively reduce inflammation and oedema.
• Combination chemotherapy is used to treat systemic or multifocal LCH, usually involving:
Prednisone or prednisolone corticosteroids; vinblastine, etoposide, or cytarabine
• 6-mercaptopurine or methotrexate for situations that are recurrent or resistant.
➣ Interventions in Nursing and Support
• Pain management: Monitoring the pain response and administering analgesics on a regular basis for acute discomfort.
Maintaining good dental hygiene involves brushing your teeth gently to lower your risk of bleeding and infection.
• Nutritional assistance: Soft food guidelines to avoid exacerbating oral lesions.
• Psychosocial care: Parental therapy for anxiety related to chronic illnesses.
•Monitoring: Consistent monitoring to track the progression of lesions and identify any systemic dissemination to organs or bones.
Notes
for more details visit 10.4103/jomfp.JOMFP_10_17