Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Case Report
2007:73:2;114-116
doi: 10.4103/0378-6323.31899
PMID: 17456920

Langerhans cell histiocytosis of the mandible in a six-year-old child

Pratibha Ramani1 , T Chandrasekar1 , Mirza F Baig2 , MR Muthusekar2 , Salim Thomas3 , Ravikanth Manyam1 , MS Senthil2
1 Department of Oral and Maxillofacial Pathology, Saveetha Dental College and Hospitals, Chennai, Tamil Nadu, India
2 Department of Oral and Maxillofacial Surgery, Saveetha Dental College and Hospitals, Chennai, Tamil Nadu, India
3 Saveetha Dental College and Hospitals, Chennai, Tamil Nadu, India

Correspondence Address:
Pratibha Ramani
C/O Dr.V.R. Janaki, #3, Sri Ram Bhavan, 19, 3rd street, Abhiramapuram, Chennai-18, Tamil Nadu
India
How to cite this article:
Ramani P, Chandrasekar T, Baig MF, Muthusekar M R, Thomas S, Manyam R, Senthil M S. Langerhans cell histiocytosis of the mandible in a six-year-old child. Indian J Dermatol Venereol Leprol 2007;73:114-116
Copyright: (C)2007 Indian Journal of Dermatology, Venereology, and Leprology

Abstract

Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis entities, may be solitary or multiple. Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. Spontaneous regression of localized disease has also been reported. We report a six and a half-year-old patient with Langerhans cell histiocytosis - solitary eosinophilic granuloma of the mandible that initially regressed but rapidly recurred even after radical treatment and had a fatal outcome.
Keywords: Birbeck granules, Langerhans cell, S 100
Birbeck granules with terminal bulging giving a characteristic tennis racquet appearance
Birbeck granules with terminal bulging giving a characteristic tennis racquet appearance
Orthopantomogram revealed multilocular osteolytic lesion of the left mandible with fl oating teeth
Orthopantomogram revealed multilocular osteolytic lesion of the left mandible with fl oating teeth
Diffuse swelling of the left mandible
Diffuse swelling of the left mandible

INTRODUCTION

Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Lichtenstein first suggested it in 1953. [1] Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis (LCH) entities might be solitary or multiple. It occurs most often in young adults and children. As many as half the patients with solitary lesions are younger than 10 years. [2] Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. [3] Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. [4] Spontaneous regression of localized disease has also been reported. [5]

We report a patient of Langerhans cell histiocytosis - solitary eosinophilic granuloma of the mandible that initially regressed but rapidly recurred even after radical treatment and ultimately had a fatal outcome.

CASE REPORT

A six and a half-year-old girl was referred to the Department of Oral and Maxillofacial Surgery with the complaint of pain and swelling in the left lower jaw. The swelling was noticed a month back and an incisional biopsy was suggestive of fibroxanthoma.

On examination, a single, diffuse swelling measuring about 6 x 6 cm in size extending beyond the lower border of the left mandible was noted [Figure - 1]. On palpation, the swelling was tender, hard, immobile and attached to the underlying structures. Intraoral examination revealed a proliferating mass with both buccal and lingual expansion extending between deciduous left canine to the retromolar region of the mandible with well-defined margins. The submandibular lymph nodes were palpable, tender and mobile. The clinical differential diagnosis included reticuloendotheliosis, intraosseous sarcoma and hyperparathyroidism.

An orthopantomogram revealed a multilocular, osteolytic lesion of the left mandible with floating teeth [Figure - 2]. Complete hemogram showed a raised ESR. The differential leukocyte count revealed a moderately raised eosinophil count. Liver function and renal function tests were normal. An incisional biopsy was performed under local anesthesia. The specimen was friable and resembled granulation tissue. Histopathology showed proliferation of histiocytic cells and infiltration of eosinophils and neutrophils along with increased vascularity. Immunohistochemical staining showed that the histiocyte cells were positive for S100 protein. These features were consistent for eosinophilic granuloma.

To evaluate the possibility of systemic involvement, a radiographic skeletal survey and thallium 201 scintillography were done, but did not reveal any abnormality. Ultrasound of the abdomen did not reveal any organomegaly. The final diagnosis was solitary eosinophilic granuloma of the mandible.

To facilitate a peripheral ostectomy of the mandible, chemotherapy was started. Vincristine 1 mg was injected once a week for four weeks and oral prednisolone 25 mg daily was given for six weeks. On 8th July 2003, a peripheral ostectomy of the left mandible was performed under general anesthesia. The postoperative histopathology report was consistent with Langerhans cell histiocytosis. Transmission electron microscopic examination (TEM) of a small tissue sample of the postoperative specimen revealed straight or curved rod shaped granules arising from the cell membrane and growing into the cytoplasm. They showed terminal bulging, giving a characteristic tennis racquet appearance, which is diagnostic for Birbeck granules, confirming the diagnosis of Langerhans cell histiocytosis [Figure - 3]. The patient was discharged following an uneventful recovery.

The patient was rehospitalized after two months with complaints of headache, vomiting and worsening of the mandibular swelling with purulent discharge from it. Culture of pus from the swelling did not grow any organisms. Raised intracranial tension was clinically suspected but a CT scan of the brain (with contrast) ruled this out. The patient was given antibiotics, analgesics, anti-emetic drugs and intravenous/oral fluids. A subsequent radiograph showed a pathologic fracture with recurrence of the lesion. Injection ciprofloxacin 100 mg twice daily and injection metronidazole 100 mg twice daily with intravenous fluids was administered for five days, followed by mandibular resection. The histopathology and electron microscopy of the resected specimen confirmed LCH.

The patient was placed under intensive medical care as she was progressively losing weight and her general condition was deteriorating. However, she finally succumbed within twenty days to the disease.

DISCUSSION

Treatment of LCH of the jaws poses many problems, as shown by the history of our patient. Our patient clinically, radiographically as well as histopathologically had the features of Langerhans cell histiocytosis - solitary eosinophilic granuloma.

Along with Birbeck granules, LCH lesions may also contain lysosomes [6] as seen in our case in TEM. These lysosomes are said to be activated in response to steroids and causes apoptosis following radiation and chemotherapy [7] and does not indicate the reason for recurrence of the lesion.

The common side effects after chemotherapy including nausea, vomiting, hair loss, increased risk of infection, tiredness and diarrhea are very unusual, as mild treatment is generally recommended for LCH. A few children may develop recurrence many years later. [8] However, our patient had recurrence within one month of initial treatment. A hazard ratio of 7.1 and 11.6 for reactivation of LCH after initial treatment, implicating higher chances in multiple organ and bone involvement than solitary lesions. [9] Thus the chances for recurrence are higher in patients with multiple lesions when compared with patients with solitary lesion.

Langerhans cell histiocytosis - eosinophilic granuloma may be a benign, self-limited disease. However, in very rare instances, a solitary LCH may recur rapidly within a short duration and lead to a fatal outcome as seen in our patient.

References
1.
Lichtenstein L. Histiocytosis X: Integration of eosinophilic granuloma of bone, Letterer-Siwe disease and Schuller-Christian disease as related manifestations of a single nosologic entity. Arch Pathol 1953;56:84-102.
[Google Scholar]
2.
Gnanasekar JD, Ahmed MS, Reddy RR. Multifocal langerhans cell histiocytosis of the jaws: A case report. Quintessence Int 1991;22:559-64.
[Google Scholar]
3.
Ardekian L, Peled M, Rosen D, Rachmiel A, Abu el-Naaj I, Laufer D. Clinical and radiographic features of eosinophilic granuloma in the jaws: Review of 41 lesions treated by surgery and low-dose radiotherapy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:238-42.
[Google Scholar]
4.
Roychoudhury A, Shah N, Parkash H, Mukhopadhyay S, Chopra P. Eosinophilic granuloma of the jaws. Br J Oral Maxillofac Surg 1998;36:380-3.
[Google Scholar]
5.
Broadbent V, Pritchard J, Davies EG, Levinsky RJ, Heaf D, Atherton DJ, et al. Spontaneous remission of multi-system histiocytosis X. Lancet 1984;1:253-4.
[Google Scholar]
6.
Miser JS, Pappo AS, Triche TJ, Merchant TE, Rao BN. Other soft tissue sarcomas of childhood. In: Pizzo PA, Poplack DG, editors. Principles and practice of pediatric oncology. 4th ed. Philadelphia: Lippincott Williams and Wilkins; 2002. p. 302.
[Google Scholar]
7.
Ball A, Baratt GM, Wills ED. Activation of lysosomal enzymes and tumour regression caused by irradiation and steroid hormones. Eur J Cancer Clin Oncol 1982;18:489-94.
[Google Scholar]
8.
Schepman KP, Radden BG, Van der Waal I. Langerhans' cell histiocytosis of the jaw bones: Report of 11 cases. Aust Dent J 1998;43:238-41.
[Google Scholar]
9.
Predictors of outcome in children with Langerhan cell Histiocytosis. ASG, Annual meeting proceedings (post meeting edition). J Clin Oncol 2004;22:8550.
[Google Scholar]

Fulltext Views
304

PDF downloads
135
Show Sections