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Table of Contents   
CASE REPORT  
Year : 2014  |  Volume : 25  |  Issue : 2  |  Page : 228-230
Oral manifestion of Langerhans cell histiocytosis mimicking inflammation


Department of Pedodontics, Educare Institute of Dental Sciences, Kottakkal, Kerala, India

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Date of Submission18-Mar-2013
Date of Decision31-Mar-2013
Date of Acceptance23-May-2014
Date of Web Publication4-Jul-2014
 

   Abstract 

Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells. LCH affects five children per million population. The peak incidence is from 1 to 4 years of age. LCH involves the head and neck region quite commonly. Oral soft tissue lesions are also common. The differential diagnosis of oral LCH includes leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. The prognosis of LCH depends on early detection and appropriate management. Surgical management alone is used in 50% of cases with an additional 23% of the lesions being treated with both surgery and radiation therapy. A case of LCH in a 6-year-old girl involving the mid root level of developing first permanent molar with a floating developing tooth bud of permanent second molar mimicking an inflammation is reported.

Keywords: Eosinophilic granuloma, histiocytosis X, inflammation, Langerhans cell histiocytosis

How to cite this article:
Divya K S. Oral manifestion of Langerhans cell histiocytosis mimicking inflammation. Indian J Dent Res 2014;25:228-30

How to cite this URL:
Divya K S. Oral manifestion of Langerhans cell histiocytosis mimicking inflammation. Indian J Dent Res [serial online] 2014 [cited 2019 Nov 12];25:228-30. Available from: http://www.ijdr.in/text.asp?2014/25/2/228/135930
Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells, which are freely mobile cells that originate from bone marrow myeloid precursors. [1] In 1868, Paul Langerhans discovered epidermal dendritic cells and the conditions, which were characterized by these cells were subsequently called different names like Hand-Schuller-Christian disease, Letterer-Siwe disease, and eosinophilic granuloma. Farber (1941) suggested that these three different entities were part of the same disease process with variation in severity, site, and stage. Lichenstein and Jaffe in 1953 included all these entities under the general category of Histiocytosis X. The unification of these three separately described diseases was based on identical histopathologic features of the lesions, which were composed of eosinophils, histiocytes, and lymphocytes. The original disease designations, Hand-Schuller-Christian disease, Letterer-Siwe disease, and eosinophilic granuloma, are still used by some practitioners when referring to the clinical features of LCH. [2]

Langerhans cell histiocytosis involves the head and neck region quite commonly, and in particular, the bones of the skull and jaws. Oral soft tissue lesions are also common and most often found with the gingiva and hard palate. [2] A case of LCH involving the mid root level of developing first permanent molar with a floating developing tooth bud of permanent second molar mimicking an inflammation is reported.


   Case report Top


A 6-year-old girl was referred to the outpatient department of pedodontics, the patient had consulted a private clinic with a 1 month history of pain and swelling of the left lower jaw [Figure 1]. There she underwent the removal of badly decayed teeth. Two weeks later, she was referred to us as the swelling persisted even after the procedure.
Figure 1: Swelling on the left lower jaw

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Examination revealed an extra-oral swelling (1.5 × 1.5 cm) on left side of the face obliterating the angle of mandible. The swelling was smooth, firm and nontender. The overlying skin was intact and slightly erythematous. The swelling was nonpulsatile, nonfluctuant and no thrill was elicited. On intra oral examination the region showed a healed extraction site with obliteration of the buccal sulcus. There was also a hard and nonfluctuant lesion extending distally from healed extraction site to retromolar pad area. Examination showed no signs of inflammation.

An orthopantomograph revealed a multilocular radiolucency with irregular margins. The developing tooth bud of permanent second molar was found to have a floating appearance [Figure 2]. The lesion was found to start at the mid root level of developing first permanent molar. The extraction site showed no relevant radiographic abnormality. The radiograph revealed relevant information to plan a surgical correction.
Figure 2: Orthopantomogram showing multilocular radiolucency with irregular margins and floating second molar

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Further a general skeletal radiograph survey revealed no abnormality. The patient had a normal blood profile and her liver function test, coagulation profile, and urine analysis was normal. Fine-needle aspiration cytology showed proliferation of histiocytic cells and infiltration of eosinophils and neutrophils along with increased vascularity. Tissue cytology revealed excessive proliferation of histiocytes suggestive of LCH [Figure 3]. Immunohistochemistry though a more diagnostically specific test, it was not performed due to nonavailability of the same. Based on clinical, radiographic and histopathology findings a diagnosis of LCH was made, and the patient was referred to the regional cancer center for further treatment. The patient was managed using surgical curettage and bone grafting. Patient is currently normal and is being reviewed both clinically and radiographically at 6 months intervals at the cancer centre.
Figure 3: Slide showing excessive proliferation of histiocytes

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   Discussion Top


Langerhans cell histiocytosis is a rare disease that affects five children per million population. The peak incidence is from 1 to 4 years of age. [2] The age of onset in this case being 6 years falls outside the peak incidence age. The most common presenting symptom is local bone pain (41%). Oral mucosal lesions although infrequent, are characterized by gingival hypertrophy and ulcers of the buccal mucosa, hard and soft palates, and tongue. [3] Oral involvement may predate systemic manifestations or mimic infectious/inflammatory disorders. [3],[4] This case represents an oral manifestation mimicking an inflammatory pathology, with a febrile illness and oral swelling as the presenting symptoms. Extra-oral signs and symptoms are quite common. The skull and lower extremities are the most common extra-oral sites of bony involvement. [2] Oral manifestation may predate systemic involvement is well illustrated in this case by her normal general skeletal survey."

Langerhans cell histiocytosis should be looked for in children presenting with severe bone loss in primary dentition. Differential diagnoses include leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. [5] Acute myeloid leukemia may present with gingival hypertrophy and appear as LCH. Gingivitis, periodontal disease and the loss of permanent teeth is a common problem across the spectrum of neutropenic syndromes. These problems can be attributed to a failure in the natural function of neutrophils which normally migrate to the gingival crypts where bacteria tend to proliferate. Prepubertal periodontitis is essentially periodontitis of primary teeth, and may include gingival inflammation, early loss of primary teeth, and bone loss. Prepubertal periodontitis usually associated with a child who has some kind of underlying medical condition, including but not limited to neutropenia, hypophosphatasia, Papillon-Lefevre syndrome, and acrodynia. However, it can also occur in otherwise healthy children, although they do not suffer from bone or attachment loss. [5] The craniofacial type of fibrous dysplasia in which only the craniofacial complex are involved including the jaw and maxilla. The craniofacial bones are more affected in the polyostotic (50-100%) than in the monostotic form (20%). Fibrous dysplasia of the jaws affects the maxilla more frequently than the mandible and affects females more frequently than males. Papillon-Lefevre syndrome is associated with marked destruction of alveolar bone with premature primary tooth loss. This condition is differentiated from LCH by the associated hyperkeratosis of the palms and soles. Hypophospatasia is characterized by low serum alkaline phosphatase levels and excessive excretion of phosphoethanolamine in the urine. The classic oral finding of hypophosphatasia is premature tooth loss generally involving the mandibular primary incisors, which often have abnormally large pulp spaces. [6]

The prognosis of LCH as with any other disease depends on early detection. When considering the fact that LCH can often present initially with an oral lesion, the knowledge about the oral manifestations can greatly reduce the morbidity and mortality of this disease. Surgical management alone is used in 50% of cases with an additional 23% of the lesions being treated with both surgery and radiation therapy. [7] Radiation alone is used in one fifth of affected individuals. Chemotherapy and intralesional steroid injection are the other modes employed. Surgery alone has a 12% recurrence rate, compared with 25% for radiation therapy alone and 19% for combined surgery and radiation therapy. However, additional radiation is best avoided in children, if it can be done without. Localized and isolated mandibular lesions may be efficiently treated by surgical curettage. When surgery leaves large bony defects, autologous bone grafts can be made in an attempt to reduce the risk of pathological fracture and to facilitate bone regeneration. [7] Survival in oral and maxillofacial LCH is quite favorable with only 7% mortality. [2] This case was treated successfully with surgical curettage alone as there was an isolated lesion with no systemic involvement and good prognostic indicators. Because future tooth development is often altered or residual defects may remain after the disease has been arrested, orthodontic guidance and prosthetic care may be essential. It is therefore important to prepare and execute a plan to guide the child's growth and development through maturity to adulthood so that the effects of necessary interventions can be reduced to a minimum. This case highlights the need for a broader outlook while dealing with inflammatory conditions. The lytic lesions need be screened with the view that rarer conditions might cause them. The inflammatory mimic if not identified correctly can lead to excess morbidity and probable mortality. This case also stresses the need for pediatricians and pediatric/general dentist to be aware of LCH mimicking oral inflammation.

 
   References Top

1.Coppes-Zantinga A, Egeler RM. The Langerhans cell histiocytosis X files revealed. Br J Haematol 2002;116:3-9.  Back to cited text no. 1
    
2.Hicks J, Flaitz CM. Langerhans cell histiocytosis: Current insights in a molecular age with emphasis on clinical oral and maxillofacial pathology practice. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100 2 Suppl: S42-66.  Back to cited text no. 2
    
3.García de Marcos JA, Dean Ferrer A, Alamillos Granados F, Ruiz Masera JJ, Barrios Sánchez G, Romero Ortiz AI, et al. Langerhans cell histiocytosis in the maxillofacial area in adults. Report of three cases. Med Oral Patol Oral Cir Bucal 2007;12:E145-50.  Back to cited text no. 3
    
4.Glotzbecker MP, Carpentier DF, Dormans JP. Langerhans cell histiocytosis: Clinical presentation, pathogenesis, and treatment from the LCH etiology research group at the children's hospital of Philadelphia. Univ Pa Orthop J 2002;15:67-73.  Back to cited text no. 4
    
5.Shafer WG, Hine MK, Levy BM. Oral aspects of metabolic disease. In: Textbook of Oral Pathology. 4 th ed. WB Saunders Publishing company, Philadelphia; 1997. p. 633-45.  Back to cited text no. 5
    
6.Kamen S, Crespi P, Eisenbud L, Dolan T. Papillon-Lefevre syndrome: Pediatric dental management. J Pedod 1986;10:356-64.  Back to cited text no. 6
[PUBMED]    
7.Bartnick A, Friedrich RE, Roeser K, Schmelzle R. Oral Langerhans cell histiocytosis. J Craniomaxillofac Surg 2002;30:91-6.  Back to cited text no. 7
    

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Correspondence Address:
K S Divya
Department of Pedodontics, Educare Institute of Dental Sciences, Kottakkal, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9290.135930

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