lch oral mucosa

8/9/2019 LCH Oral Mucosa

1/4

76

Histiocytosis is a term applied to a group of rare disorders

of the reticuloendothelial system. Though these disorders

present with different clinical features, they have anumber of histologic features in common, including the

abnormal proliferation of histiocytic cells, which have

been shown to represent Langerhans cells. These

diseases are thus collectively known asLangerhans cell

histiocytosis (LCH), formerly histiocytosis X.1

The etiopathogenesis of LCH is not clear. Although

LCH might represent a reactive response to a defect in

cell-mediated immunity,2 recent clonality studies have

suggested that this is a neoplastic disorder. Bone, lungs,

liver, lymph nodes, spleen, hematopoietic tissue, and

mucocutaneous tissues might all be affected, thereby

generating a marked variability in clinical presentation.3

The severity and prognosis of the disease is in turn

dependent on the type and extent of organ involvement.4

Bone is the tissue most frequently affected; the

majority of patients present with solitary or multiple

bone lesions, and involvement of the jaw (particularly

the mandible) is not unusual.5 Oral mucosal involve-

ment is much less frequent and typically is associated

with lesions of the underlying bone.6

We present two patients with oral mucosal ulceration

confirmed by histologic and immunohistochemical

study to be LCH without any evidence of involvement

of bone or other organs.

CASE REPORTSCase 1

A 50-year-old man presented with painless palatal ulcera-

tion of 3 months duration. He gave a history of urolithiasis,

hiatal hernia, and diabetes mellitus treated with oral hypo-

glycemic medication. The patient smoked cigarettes, was

overweight, and consumed a moderate amount of alcohol.

Examination showed a 1.0-cm ulceration of the hard palate,

adjacent to the maxillary right second molar. The lesion hadnonindurated irregular margins and a clean base (Fig 1). The

related teeth and periodontium showed no significant

pathosis. Neither exophthalmos nor diabetes insipidus was

observed.

Orthopantomography showed no bone, dental, or maxillary

sinus disease. Computed axial tomography also failed to

detect bone lesions.

Laboratory evaluation demonstrated that the patient had

increased blood levels of glucose (316 mg %), uric acid (8.5

mg/dL), cholesterol (336 mg/dL), and triglyceride (1540

Langerhans cell histiocytosis restricted to the oral mucosa

M. Angeles Milin, MD, PhD, MDS,a Jos V. Bagn, MD, PhD, MDS,b Yolanda Jimnez, MD,

PhD, MDS,a Ana Prez, MD,c Crispian Scully, MD, PhD, MDS, MRCS, FDSRCS, FDSRCPS,

FFDRCSI, FDSRCSE, FRCPath, FmedSci,d and Dimitris Antoniades, DDS,e Valencia, Spain;

London, United Kingdom; and Thessaloniki, Greece

VALENCIA UNIVERSITY, EASTMAN DENTAL INSTITUTE OF LONDON, AND DENTAL SCHOOL OF THESSALONIKI

Langerhans cell histiocytosis comprises a group of disorders with Langerhans cell proliferation as a commonfeature. The clinical presentation might be highly varied. Typically, there is bone involvement and, less frequently, lesions

might be found in other organs, particularly the lungs, liver, lymph nodes, skin, and mucosae. Lesions limited to the oralmucosa are rare. We describe two patients with unifocal eosinophilic granuloma exclusively limited to the oral mucosa.Triamcinolone acetonide infiltration of a palatal lesion yielded a good result in one patient. Six months later, a similar lesiondeveloped in the mandibular gingival mucosa, but it responded to the same treatment. The lesion in the other patientresponded to local radiotherapy. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;91:76-9)

a

Associate Professor of Oral Medicine, Valencia University, Spain.bFull Professor of Oral Medicine, Valencia University, Head of the

Service of Stomatology, Valencia University General Hospital, Spain.cResident, Service of Pathology, Valencia University General

Hospital, Spain.dEastman Dental Institute, London, United Kingdom.eDental School, Thessaloniki, Greece.

Received for publication Aug 14, 1999; returned for revision Dec 14,

1999, and Apr 13, 2000; accepted for publication Jul 6, 2000.

Copyright 2001 by Mosby, Inc.

1079-2104/2001/$35.00 + 0 7/14/110031

doi:10.1067/moe.2001.110031

Fig 1. Case 1: Clinical appearance of ulceration on the hard

palate.


2/4

ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Milin et al 77Volume 91, Number 1

mg/dL). The Mantoux test and the human immunodeficiency

virus and hepatitis B virus serology were negative.

A routine hematoxylin-neosinstained histologic section

revealed a mixed inflammatory infiltrate mainly consisting of

cells with a histiocytic appearance (Fig 2). S-100 protein anti-

body labeling showed that these were consistent with

Langerhanscells. Polymerase chain reaction excluded herpes

simplex and cytomegalovirus infections.

These findings were considered to be consistent with LCH

(Fig 3). Multifocal LCH was ruled out because chest radio-

graphs and liver function studies showed normal results, and

a gammagraphic bone scan showed no evidence of otherbone lesions.

In view of the above findings, a final diagnosis of LCH

exclusively limited to the oral mucosa was established.

Perilesional triamcinolone acetonide infiltration (25 mg: 1

injection every 3 weeks for a total of 8 sessions) was

performed and the lesion resolved completely. Additional oral

mucosal lesions later developed on the edentulous alveolar

ridge in the mandibular right posterior quadrant, appearing

similar to the palatal lesion, though more shallow. Again,

there were no evident systemic lesions, and resolution with

intralesional corticosteroids was noted. No new lesions have

been observed after 1 year of follow-up.

Case 2A 52-year-old woman presented with painful ulceration (of

2 months duration) lingual to the mandibular left first molar,

with no other complaints and an unremarkable medical history.

Examination showed a 1-cm diameter discrete ulcer with no

cervical lymph node involvement (Fig 4). Radiographs showed

no jaw involvement. A biopsy specimen showed a light micro-

scopic and immunohistochemical pattern consistent with LCH

(Figs 5, 6, and 7). No systemic lesions were detectable by

physical examination, chest and skull radiographs, or tech-

netium diphosphonate bone scan, and thus a diagnosis of local-

ized LCH was made.

The patient was treated successfully with radiotherapy to

the lesion, and after 3 years of follow-up, there was neither

recurrence nor evidence of lesions elsewhere.

DISCUSSION

LCHis the term used to describe a group of disorders

histologically characterized by the proliferation of cells

Fig 2. Case 1: Histologic appearance of the lesion (hema-

toxylin-eosin).

Fig 3. Case 1: Immunohistochemical results, showing S-100

protein positivity of lesional cells.

Fig 4. Case 2: Clinical appearance of gingival ulceration.

Fig 5. Case 2: Low-power histologic appearance of the

lesion (hematoxylin-eosin).


3/4

78 Milin et al ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGYJanuary 2001

having a histiocytic appearance. The Langerhans cellorigin has been demonstrated by immunoreactivity of

the lesional cells for S-100 protein, HLA-DR, and

CD1a, and on electron microscopy by the presence of

typical Birbeck granules.1

LCH comprises Letterer-Siwe disease (acute dissem-

inated histiocytosis), Hand-Schller-Christian disease

(chronic disseminated histiocytosis), and eosinophilic

granuloma (monostotic and polyostotic). The clinical

manifestations of these 3 types of LCH differ consider-

ably, and the prognosis ranges from very poor in thecase of Letterer-Siwe disease to good in the case of

eosinophilic granuloma.

Eosinophilic granuloma tends to affect younger adults;

individuals over the age of 50 are uncommonly affected.

Eosinophilic granuloma particularly involves bone, most

commonly the skull, femur, pelvis, mandible, ribs, tibia,

clavicle, scapula, spine, or humerus. Soft tissue involve-

ment might occur, however, affecting mainly the lymph

nodes, lungs, and mucous membranes.7 Oral mucosal

Fig 6. Case 2: Medium-power histologic appearance of the lesion (hematoxylin-eosin).

Fig 7. Case 2: High-power histologic appearance of the lesion (hematoxylin-eosin).


4/4

lch oral mucosa

Documents