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Table of Contents   
SHORT COMMUNICATION  
Year : 2012  |  Volume : 23  |  Issue : 6  |  Page : 830-832
Hand-Schüller-Christian disease


1 Department of Oral Pathology and Microbiology, School of Dental Sciences, Sharda University, Greater Noida, India
2 Department of Oral Pathology and Microbiology, Inderprastha Dental College and Hospital, Ghaziabad, India
3 Department of Oral Pathology and Microbiology, Government Dental College and Hospital, Nagpur, India

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Date of Submission19-Oct-2010
Date of Decision14-Mar-2012
Date of Acceptance28-Apr-2012
Date of Web Publication3-May-2013
 

   Abstract 

Langerhan cell histiocytosis, formerly known as histiocytosis X, traditionally denotes a group of diseases that stem from proliferative reticuloendothelial disturbances.The etiology and pathogenesis of the disease remain debatable. In this paper we report a case of Langerhan cell histiocytosis in a 3 1 / 2 -year-old child who reported to the department of oral pathology with gingival enlargements. The radiological features and histopathological features are also discussed.

Keywords: Exophthalmos, diabetes insipidus, histiocytosis, histiocytosis x, Langerhan cell

How to cite this article:
Bhargava D, Bhargava K, Hazarey V, Ganvir SM. Hand-Schüller-Christian disease. Indian J Dent Res 2012;23:830-2

How to cite this URL:
Bhargava D, Bhargava K, Hazarey V, Ganvir SM. Hand-Schüller-Christian disease. Indian J Dent Res [serial online] 2012 [cited 2019 Aug 18];23:830-2. Available from: http://www.ijdr.in/text.asp?2012/23/6/830/111273
Langerhan cell histiocytosis (LCH), formerly known as histiocytosis X, is currently considered as a disorder of immune regulation manifested by abnormal proliferation of histiocytes and granuloma formation. The Langerhan cell, a unique histiocyte, is the distinctive pathologic component of the disease. LCH may affect any organ, although the reticuloendothelial system (i.e. lymph nodes, liver, and spleen) is involved in most cases. [1]

Langerhan cell disease manifests in three forms

  • Acute disseminated form: Previously referred to as Letterer-Siwe disease, it most likely represents a malignant neoplastic process. It is characterized by a rapidly progressive clinical course and widespread involvement of organs, bones, and skin.
  • Chronic disseminated form: Previously referred to as Hand-Schüller-Christian syndrome, it is characterized by the classical triad of lytic bone lesions, exophthalmos, and diabetes insipidus.
  • Chronic localized form: Previously termed as eosinophilic granuloma, it is characterized by only unifocal or multifocal bone lesions. [2]
The pathogenesis of LCH is unknown. It may be due to a hypersensitivity reaction to an unknown antigen, with stimulation of the histiocyte-macrophage system. Deficiency of suppressor lymphocytes (T 8 ), altered immunoglobulin's autoantibodies, and structural changes in the thymus (in all the advanced forms) have been found in LCH patients. An inflammatory origin is also suspected due to the microscopic characteristics and the clinical evolution of the disease. [3]

Here, we report a case of chronic disseminated Langerhan cell disease (Hand-Schüller-Christian disease) that presented with gingival enlargement.


   Case Report Top


A 31/2 -year-old male child was referred to the department of oral pathology with pain and swelling in the deciduous canine and first molar region, involving all the four quadrants. The symptoms had been present for 5 months, and there was associated mobility of the deciduous canines and first molars. The Child had history of oral ulcers and had intolerance to hot Food. There was no history of trauma and family history was non-contributory. General examination of the patient by a pediatrician and an ENT surgeon revealed the presence of hepatosplenomegaly and otitis media [Figure 1].
Figure 1: Preauricular pus discharge, suggestive of otitis media

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Extraoral examination revealed right submandibular lymphadenopathy, There was no exophthalmos. Intraorally, gingival enlargement was seen in the molar region in all the four quadrants; in each area the swelling was approximately 1.5 × 2 cm in size, reddish in color, and soft to firm in consistency. Bleeding was evident on the slightest provocation [Figure 2].
Figure 2: Intraoral photograph shows gingival enlargement

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Radiographic examination showed punched-out lytic lesions in the skull, involving the parietal, temporal, and frontal bones. In the jaws there was diffuse destruction of bone and displacement of teeth [Figure 3]. Lab investigations, which included complete blood count, liver function tests, coagulation studies, and urine osmolality, were within normal limits.
Figure 3: Radiograph (lateral view) shows multiple punched-out lesions in skull

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Microscopic examination of the lesion revealed the presence of many histiocyte-like cells, eosinophils, and lymphocytes, diffusely scattered throughout the connective tissue stroma. In view of the clinical, radiological, and histopathological features, we diagnosed Langerhan cell disease (histiocytosis X), in particular the chronic disseminated form or Hand-Schüller-Christian disease [Figure 4] and [Figure 5].
Figure 4: Hematoxylin and eosin-stained section shows numerous histiocyte-like cells, eosinophils, and lymphocytes scattered in a loose connective tissue stroma (10×)

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Figure 5: Hematoxylin and eosin-stained section shows numerous histiocyte-like cells, eosinophils, and lymphocytes scattered in a loose connective tissue stroma (40×)

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The patient was put on prednisolone 5 mg, four times a day, for 3 weeks. Supportive treatment included antibiotics and eardrops. On subsequent follow-up, the patient had ear discharge and swelling in the frontal and parietal region of Skull and was advised bone biopsy. Biopsy report confirmed histiocytosis X and the patient was advised to continue the same treatment and was recalled for periodic review. However, the patient did not report for subsequent follow-up.


   Discussion Top


Hand-Schüller-Christian disease is characterized by widespread skeletal and extraskeletal lesions and a chronic clinical course. It occurs usually before the age of 5 years but has been reported even in young adults. It is more commonly seen in boys, with male:female ratio of approximately 2: 1. In Hand-Schüller-Christian disease the skeletal system and soft tissues may be involved, while in eosinophilic granuloma only the bone is affected, although soft tissue extension is often observed. Letterer-Siwe disease is an acute fulminating disease with widespread lesions of both skeletal and extraskeletal tissues, including the skin. [4]

Hand-Schüller-Christian disease is characterized by the classic triad of single or multiple punched-out bone lesions in the skull, unilateral or bilateral exophthalmos, and diabetes insipidus, with or without other manifestations like polyuria, dwarfism, or infantilism. The complete triad is however seen only in 25% of affected patients. Involvement of facial bones is frequently associated with soft tissue swelling, tenderness, and facial asymmetry. Otitis media is also common. Other bones that are frequently involved are femur, ribs, vertebrae, and pelvis. Sometimes, the skin exhibits papular or nodular lesions. [4]

Oral manifestations are the earliest signs in around 5%-75% of patients. These include sore mouth, halitosis, gingivitis, unpleasant taste, loose teeth, and failure of extracted tooth sockets to heal. Loss of supporting bone mimics advanced periodontal disease. Radiographic examination reveals that individual lesions, particularly in the skull, are sharply outlined. On the other hand, those in the jaws may be more diffuse, exhibiting destruction of alveolar bone with tooth displacement. [4]

Histologically, Hand-Schüller-Christian disease manifests four stages during its progression:

  • A proliferative histiocytic phase, with collections of eosinophilic leukocytes scattered throughout the sheets of histiocytes
  • A vascular granulomatous phase, with persistence of histiocytes and eosinophils; sometimes with aggregation of lipid-laden (cholesterol) macrophages
  • A diffuse xanthomatous phase, with an abundance of 'foam cells'
  • A fibrous or healing phase[4]
Ultrastructurally, Langerhan cells contain rod-shaped cytoplasmic structures known as Birbeck granules, which differentiate Langerhan cells from other mononuclear phagocytes. Langerhan cells show immunoreactivity to CD-1a or CD-207, the latter marker being especially specific to Langerhan cells. In a few cases, lesional cells have shown immunoreactivity to S-100 protein and peanut agglutinin (PNA). [5] Lab investigations often reveal anemia, leukopenia, and thrombocytopenia. The serum cholesterol level is nearly normal, though tissue cholesterol content may be elevated remarkably. [4]

The treatment of choice is curettage or excision of lesions. Inaccessible lesions may be irradiated. Some patients are benefited from chemotherapeutic drugs, including prednisolone. One of the significant factors influencing the morbidity and mortality of the disease is the extent of the disease at the time of initial diagnosis and the number of organ systems involved. [4]

 
   References Top

1.Stull M, Kransdorf MJ, Devaney KO. Langerhans cell histiocytosis of Bone. Radiographics 1992;12:801-23.  Back to cited text no. 1
    
2.Jayachandran S, Murali Gopika Manoharan GV. Langerhans cell disease: A case report. J Ind Acad Oral Med Radiol 1999;10:9-13.  Back to cited text no. 2
    
3.Madrigal-Martinez-Pereda C, Guerrero-Rodriguez V, Guisado-Moya B, Meniz-Garcia C. Langerhans cell histiocytosis: Literature review and descriptive analysis of oral manifestations. Med Oral Pathol Oral Cir Buccal 2009;14:222-8.  Back to cited text no. 3
    
4.Shafer WG, Hine MK, Levy BM. Diseases of bone and joints. In: Rajendran R, Shivapathasundaram B, editors. Shafer's textbook of oral pathology. 6 th ed. Noida: Elsevier; 2009. p. 744-5.  Back to cited text no. 4
    
5.Neville BW, Damm DD, Allen CM, Bouquot JE. Langerhans cell histiocytosis. Textbook of oral and maxillofacial pathology. 3 rd ed. New Delhi: Elsevier; 2009. p. 590-2.  Back to cited text no. 5
    

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Correspondence Address:
Deepak Bhargava
Department of Oral Pathology and Microbiology, School of Dental Sciences, Sharda University, Greater Noida
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9290.111273

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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