Langerhans Cell Histiocytosis:

Question 1

Langerhans Cell Histiocytosis:

Definition:

Answer 1

Langerhans cell histiocytosis (LCH) is an abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells), which comprise an unusual group of disorders with focal or systemic manifestations

• “Langerhans cell histiocytosis” remains the preferred nomenclature; the historical terms histiocytosis-X, Letterer-Siwe disease, Hand-Schüller-Christian disease, and diffuse reticuloendotheliosis have been abandoned

Question 2

Langerhans Cell Histiocytosis:

“Eosinophilic granuloma”:

Answer 2

The term “eosinophilic granuloma” is sometimes used to describe the pathology of an individual lesion, particularly isolated lytic processes in bone

Question 3

Langerhans Cell Histiocytosis:

The clinicopathologic spectrum traditionally considered under the designation of Langerhans cell histiocytosis includes the following:

Answer 3

• Monostotic or polyostotic eosinophilic granuloma of bone— solitary or multiple bone lesions without visceral involvement
• Chronic disseminated histiocytosis—a disease involving bone, skin, and viscera (Hand-Schüller- Christian disease)
• Acute disseminated histiocytosis—a disease with prominent cutaneous, visceral, and bone marrow involvement occurring mainly in infants (Letterer-Siwe disease)

Question 4

Langerhans Cell Histiocytosis:

Differential diagnosis:

Answer 4

Since oral lesions are frequently intraosseous and it could mimic others clinical conditions, other lesions must be considered as differential diagnosis of LCH, as: giant cell granuloma, granulomatous diseases, osteomyelitis, odontogenic tumors, certain benign and malignant neoplasms of bone, lymphomas, leukemias, multiple myeloma, metastatic disease

Question 5

Langerhans Cell Histiocytosis:

Oral lesions:

Answer 5

• The most common oral manifestations of LCH are intraoral mass, gingivitis, mucosal ulcers, and loose teeth. Pain and mass effects can derive from lesions of the bones or soft tissues. Some infants with LCH present with abnormally early tooth eruption
• LCH may manifest as single or multiple lesions. It can affect the alveolar or cortical bone and produce ulcerated mucosal lesions, lymphadenopathies, and periodontal lesions. Gingival inflammation, bleeding, recession, necrosis, dental mobility, and premature loss of teeth may also be observed
• Ulcerative or proliferative mucosal lesions or a proliferative gingival mass may develop if the disease breaks out of bone. Occasionally, this process may involve only the oral soft tissues. Lesions also can occur within the body of the mandible or maxilla, where they may simulate a periapical inflammatory condition
• Oral soft tissue lesions are most commonly found in gingiva and hard palate. The floor of the mouth, maxillary sinus, and buccal mucosa each account for less than 10% of lesions of LCH

Question 6

Langerhans Cell Histiocytosis:

Dentist’s role:

Answer 6

• Oral manifestations of LCH are relatively common, being important for general dentists to be able to identify its clinical signs
• Recognition of the clinical features of LCH is important to avoid misdiagnosis and to establish the correct treatment
• Dentists can play a vital role in the diagnosis of LCH since oral lesions may be the earliest manifestation and sometimes the only sign of the disease
• Additionally, oral and periodontal lesions may be early signs of disease reactivation when the patient has a history of a previous diagnostic of LCH
• Furthermore dentists need to give attention and proper follow-up to single oral lesions with a diagnosis of LCH because it may be part of a multisystem disease

Question 7

Langerhans Cell Histiocytosis:

Radiographically:

Answer 7