ORAL MANIFESTATIONS IN

SYSTEMIC DISEASESDr.Roohia

HEMATOLOGIC DISEASESIron-Deficiency Anemia:Oral manifestations may

include angular cheilitis, atrophic glossitis, mucosal pallor, and generalized atrophy of the oral mucosa.

The glossitis may include flattening of the papillae on the dorsum of the tongue and is often accompanied with a tenderness or a burning sensation.

This process may result in a smooth, erythematous tongue mimicking benign migratory glossitis, also known as “geographic tongue” or erythema migrans;

lesions on the tongue that are red and white, nonindurated, atrophic, but with a slightly elevated, white rim, and the site keeps changing over a period of time.

Plummer-Vinson syndrome or Paterson-Kelly syndrome is another type of irondeficiency anemia that presents with glossitis, and is generally seen in women of Scandinavian and Northern European origin

Pernicious Anemia:The oral manifestations

include burning of the tongue, lips, and buccal mucosa as well as other mucosal sites

Focal or diffuse erythema of the tongue and mucosal atrophy is noted, depending on the duration and severity of the condition. An interesting term, “magenta tongue”

Multifocal patchy erythematous zones on the tongue dorsum and lateral border in a patient with pernicious anemia

which is considered a low-risk area for development of squamous cell carcinoma

Leukemia:Oral complications of

leukemia include petechial hemorrhages of posterior hard palate and soft palate, and spontaneous gingival bleeding.

The gingival bleeding is precipitated when the platelet count falls between 10,000 and 20,000/mm3

Areas of tissue destruction, necrosis, ulceration of the palate significant finding

Diffuse oral candidiasis is often a complication

The leukemic cells may also produce a diffuse, boggy, nontender

swelling with or without ulceration, which appears as a gingival enlargement or even a tumor-like growth containing a collection of leukemic cells called granulocytic sarcoma or extramedullary myeloid tumor

An ulcerated gingival enlargement consistent with a leukemic “granulocyticsarcoma” noted on the left posterior buccal maxillary mucosa

Langerhans Cell Histiocytosis (Histiocytosis X):Langerhans cell histiocytosis (LCH) is characterized

by abnormal monoclonal proliferation of antigen-presenting Langerhans cells

Oral manifestations of LCH include irregular ulcerations of the hard palate, which may be the primary manifestation of the disease.

Gingival inflammation and ulcerated nodules, difficulty in chewing, and foul-smelling breath also occur. Large ulcerations with exposed bone, ecchymoses, gingivitis, and periodontitis followed by tooth loss are the hallmark of this condition.

Radiographically, the teeth often appear to be “floating in air” and in some cases may present with a large radiolucent region exhibiting significant bone loss.

Oral swellings or ulcerations resulting from

mandibular or maxillary bone involvement may be common, and ulceration may develop on the gingiva, palate, and floor of the mouth

Multiple Myeloma:Multiple myeloma usually involves the oral cavity in the later stages of the disease.An interesting finding is amyloid deposits in the tongue leading to macroglossia, which in some cases prompts a biopsy ultimately leading to the final diagnosis.Patient with multiple myeloma, with significant facial asymmetry of the left side andbuccal mucosal swelling intraorally extending from the left canine to the posterior molarregion.

AUTOIMMUNE CONDITIONSSjo¨ gren Syndrome:The oral manifestations in

SS are generally related to the low salivary volume and comprise dysphagia, dysgeusia, difficulty in eating and speaking, as well as an increased rate of dental caries and susceptibility to infection

xerostomia include dry, red, and wrinkled mucosa.

The tongue may appear “bald” or even “cobblestone-like” due to atrophy of the papilla.

Cracks, fissures of the tongue, redness, and cheilitis are additional findings

This increase in dental caries is especially noted around the cervical region of the teeth.23

Kawasaki Disease:Kawasaki disease, also

known as mucocutaneous lymph node syndrome

(1) edema of extremity, erythema;

(2) polymorphous exanthem;(3) bilateral conjunctival

injection; (4) erythema and strawberry

tongue; and (5)acute cervical

lymphadenopathy

The most striking oral finding includes swelling of papillae on the surface of the tongue (strawberry tongue) and intense erythema of the mucosal surfaces. The lips may be cracked, red, swollen, and often hemorrhagic and crusted

Scleroderma (Progressive Systemic Sclerosis; Hide-Bound Disease):

Deposition of extraordinary amounts of dense collagen in the tissues of the body.

When exposed to cold temperatures these patients show what is termed Raynaud phenomenon in their terminal phalanges.

Patient with scleroderma exposed to cold temperature exhibiting Raynaud phenomenon,with cyanotic fingers and palm

The lips appear pursed due to constriction of the mouth, thus making it difficult to open the mouth and resulting in what is termed microstomia, causing limited opening of mouth in about 70% of these patients

A “purse-string” appearance caused by creases and folds of skin radiating from the mouth may be noted.

Xerostomia is a consistent finding, and some of these patients have concurrent secondary SS

The tongue can lose mobility and generally has a smooth appearance

The collagen deposits invariablyresult in smoothening of the palatal rugae.

Diffuse widening of the periodontal ligament space is noted in the entire dentition

Systemic Lupus Erythematosus: