Sarcoidosis

Sarcoidosis is an idiopathic systemic disease characterized by bilateral hilar lymphadenopathy and noncaseating granulomas in the lungs. Ocular and cutaneous manifestations are common.

Sarcoidosis may involve nearly any organ system; organs involved include the liver, heart, spleen, eyes, kidneys, and lymph system. Pulmonary manifestations are the most common and include dyspnea with exertion, nonproductive cough, chest pain, wheezing and nasal congestion, and hemoptysis.

Sarcoidosis may manifest cutaneously in 5-20% of patients and is more common in African Americans. Examples of skin manifestations include alopecia, erythroderma, subcutaneous nodules, erythema nodosum, macules, papules and plaques, and lupus pernio. Papules are small with a waxy consistency, and they may appear on the trunk and around the nose and eyes. Lupus pernio is characterized by indurated purplish nodules that occur on the face of women with chronic sarcoidosis (Lisowe, 1998). Systemic annular plaques with pale centers and violaceous edges may also appear on the extremities, back, buttocks, and face (Lisowe, 1998).

Oral manifestations may include multiple, nodular, painless ulcerations of the gingiva, buccal mucosa, labial mucosae, and palate. Indistinct ulcerations or swellings do not aid in diagnosing sarcoidosis, but biopsy results reveal noncaseating granulomas surrounded by multinucleate giant cells along with lymphocytic infiltrate. Although less common, salivary gland involvement is a possibility, leading to tumorlike swellings. Heerfordt syndrome may arise if symptoms include parotid gland swelling, xerostomia, uveitis, and facial nerve palsy. Rarely, sarcoidosis may involve the tongue, with swelling, enlargement, and ulcerations. Involvement of the tongue is an uncommon manifestation, with few cases reported in the past 30 years.

Oral involvement in sarcoidosis usually manifests after systemic symptoms develop. This form is commonly a diagnosis of exclusion. Biopsy samples are used to identify noncaseating granulomas, a nonspecific finding. Other granulomatous diseases must be excluded, such as Wegener granulomatosis, Crohn disease, syphilis, or tuberculosis (Lisowe, 1998; Nagata, 1999). Treatment with systemic corticosteroids may be useful, but symptoms may resolve spontaneously or progress despite treatment. Multiple organ and progressive pulmonary involvement indicate a poor prognosis. The overall mortality rate is approximately 5% (Lisowe, 1998).

MULTISYSTEM

Amyloidosis

Amyloidosis (AL) is the deposition of amyloid proteins in body tissues leading to tissue damage. AL is classified as either primary or secondary. The former results from multiple myeloma or an idiopathic disease, while the latter is a sequela of a chronic or inflammatory disease process. These classifications are based on the type of fibrillar protein deposited. The primary form usually affects the skin, heart, tongue, and GI tract, while the secondary form, although more common, has no cutaneous manifestations.

Systemically, patients with primary amyloid light chain AL present with fatigue, weight loss, and symptoms specific to the organs involved. The kidneys and heart are the most common organs involved, demonstrating renal failure and aggressive congestive heart failure. CNS manifestations are absent, but peripheral neuropathies are common (Falk, 1997).

The most common oral manifestation of AL is macroglossia, which occurs in 20% of patients. The enlarged tongue demonstrates lateral ridging due to teeth indentation (Falk, 1997). Although pain is not usually present, enlargement, firmness, and loss of mobility are common. Grossly, the tongue may be firm and appear relatively normal or it may have yellow nodules on the lateral surface. Interference with taste has also been reported in some patients, and hyposalivation may result from amyloid deposition in the salivary glands. Submandibular swelling occurs subsequent to tongue enlargement and can lead to respiratory obstruction.

Microscopic examination of a biopsy sample from an enlarged tongue reveals the characteristic amorphous fissured appearance of amyloid in the tissues; a green negative birefringence is depicted when polarized light is shone on tissue stained with Congo red. The most common protein type deposited in the oral cavity is amyloid. The detection of AL in a patient warrants further evaluation for possible multiple myeloma. Of patients with multiple myeloma, especially those with AL, 7-20% have amyloid deposition. The presence of myeloma yields a poor prognosis for the patient.

DRUG-INDUCED CONDITIONS

Aphthous stomatitis

Pharmacologic agents are among the most common causes of recurrent aphthous lesions, commonly known as canker sores. Some common culprits include nonsteroidal anti-inflammatory drugs (NSAIDs), nicorandil, ACE inhibitors, and bisphosphonates, but any drug can potentially produce an aphthouslike reaction. These ulcerations are round, crateriform, yellowish depressions surrounded by an area of erythema. The size varies from 1-3 mm (aphthous minor, approximately 80% of cases) to larger than 1 cm (aphthous major, approximately 15% of cases). Patients may have outbreaks of multiple ulcerations at one time. These are usually quite painful but typically are self-limiting and resolve in 7-10 days for aphthous minor and 14-21 days for aphthous major lesions.