Sturge–Weber Angiomatosis

Definition Sturge–Weber angiomatosis, or encephalotrigeminal angiomatosis, is a relatively rare, sporadic congenital capillary vascular malformation typically involving areas innervated by the trigeminal nerve.

Etiology Developmental abnormality.

Clinical features The main clinical features are characterized by unilateral hemangiomas of the facial skin, oral mucosa, and leptomeninges, brain calcification, ocular disorders, epilepsy, and occasionally mild mental handicap. Oral hemangiomas have a bright red or purple color and are usually flat but may also be raised, causing tissue enlargement. Dentists and oral surgeons must be careful during tooth extraction and periodontal surgery so as to avoid bleeding complications. Facial hemangiomas have a bright red color and are asymptomatic. Facial and oral hemangiomas are usually obvious at birth. The diagnosis is usually based on clinical criteria.

Differential diagnosis Solitary hemangioma, Klippel–Trénaunay–Weber syndrome.

Treatment Laser therapy is recommended.

Sturge–Weber angiomatosis, facial hemangiomas

Sturge–Weber angiomatosis, oral hemangiomas on the alveolar mucosa

Peripheral Ameloblastoma

Definition Ameloblastoma is the most common tumor of odontogenic epithelial origin that primarily affects the jaws.

Etiology Unknown. The peripheral ameloblastoma probably arises fromdental lamina rests or from basal epithelial cells.

Clinical features Peripheral ameloblastoma is rare and accounts for about 1–2% of all ameloblastomas. It usually presents as a painless, slowgrowing, nonulcerated, sessile red mass. The size of the lesion varies between 1 and 2 cm. The posterior alveolar mucosa and the gingiva of the mandible are more frequently affected. The lesion causes little or no bone erosion. The clinical diagnosis should be confirmed by a biopsy and histopathological examination.

Differential diagnosis Pyogenic granuloma, peripheral giant cell granuloma, fibroma, squamous-cell carcinoma, extraosseous calcifying epithelial odontogenic tumor, extraosseous calcifying epithelial odontogenic cyst, odontogenic myxoma.

Treatment Local surgical excision.

Peripheral ameloblastoma, red mass on the posterior alveolar mucosa

Reiter Disease

Definition Reiter disease is an uncommon multisystemic disorder that predominantly affects young men aged 20–30 years.

Etiology The exact etiology remains unknown, although the pathogenesis is mediated by an immunological mechanism. The disease may be triggered by an infectious agent in a genetically susceptible individual.

Clinical features The main clinical manifestations are nongonococcal urethritis, cyclic balanitis, symmetrical arthritis of six to seven joints, conjunctivitis, prostatitis, cervicitis, and mucocutaneous lesions. The cutaneous manifestations appear as macular, vesicular, or pustular lesions involving mainly the palms and soles. Psoriasiform lesions, keratoderma, and nail changes are common. Oral lesions occur in 20–40% of the cases and are characterized by diffuse erythematous areas intermixed with thin whitish dots or lines and painful superficial erosions. The buccal mucosa, gingiva, palate, lips, and tongue are more frequently affected. The tongue lesions may mimic geographic tongue. The clinical diagnosis should be confirmed by biopsy and histopathological examination.

Differential diagnosis The differential diagnosis of oral lesions includes Behçet disease, erythema multiforme, geographic tongue, and drug reactions.

Treatment Systemic corticosteroids and nonsteroidal anti-inflammatory agents are the drugs of choice.

Reiter disease, diffuse erythema on the palate

Reiter disease, erythema and superficial erosions on the tongue

Infectious Mononucleosis

Definition Infectious mononucleosis is an acute, self-limited infectious disease that primarily affects children.

Etiology Epstein–Barr virus transmitted through saliva transfer.

Clinical features The oral manifestations are early and common, and consist of palatal petechiae, uvular edema, tonsillar exudate, gingivitis, and rarely ulcers. Generalized lymphadenopathy, hepatosplenomegaly, maculopapular skin rash, and sore throat are common. Prodromal symptoms such as anorexia, malaise, headache, fatigue, andlater fever occur before the clinical manifestations.

Laboratory tests Heterophile antibody tests, and other specific antibody tests (Paul–Bunnell test, monospot test).

Differential diagnosis Leukemias, secondary syphilis, diphtheria, fellatio,thrombocytopenic purpura, traumatic hematoma.

Treatment Symptomatic.

Infectious mononucleosis: petechiae on the palate

Thrombocytopenic Purpura

Definition Thrombocytopenic purpura is a hematological disorder characterized by a decrease in platelets in the peripheral blood.

Etiology Presumably a nonspecific viral infection, myelotoxic agents.

Clinical features The oral manifestations consist of red lesions in the form of petechiae, ecchymoses, or even hematomas, usually located on the palate and buccal mucosa. Spontaneous gingival bleeding is a constant early finding. Purpuric skin rash, epistaxis, and bleeding from the gastrointestinal and urinary tract are common.

Laboratory tests Peripheral platelet count, bone-marrow aspiration, bleeding and clotting times.

Differential diagnosis Aplastic anemia, leukemias, polycythemia vera, agranulocytosis, macroglobulinemia, drug reactions.

Treatment Steroids, platelet transfusions, cessation of drug treatment if it is drug-related.

Idiopathic thrombocytopenic purpura: petechiae and ecchymoses of the buccal mucosa

Anemia

Pernicious anemia, iron deficiency anemia, and Plummer–Vinson syndrome usually affect the oral mucosa. The oral manifestations are early and common, and are characterized by an atrophic, smooth and red tongue. A burning sensation, taste loss, angular cheilitis, and rarely erosions may be present. The differential diagnosis includes atrophic lichen planus and malnutrition disorders. The diagnosis is based on hematological laboratory tests.

Pernicious anemia: red and smooth dorsum of the tongue

Plummer–Vinson syndrome: redness and atrophy of the lingual papillae, associated with angular cheilitis

Hereditary Hemorrhagic Telangiectasia

Definition Hereditary hemorrhagic telangiectasia, or Osler–Rendu–Weber disease, is a rare mucocutaneous disorder characterized by dysplasia of the capillaries and small vessels.

Etiology Inherited as an autosomal dominant trait.

Clinical features The oral mucosa is frequently involved and the lesions present as multiple bright red papules, 1–2 mm in size, which disappear on pressure froma glass slide. Nodules and spiderlike lesions may also be seen. Hemorrhage is common after minimal mechanical damage. The lips, tongue, buccal mucosa, and palate are most frequently involved. Epistaxis and gastrointestinal bleeding are common.

Laboratory tests Histopathological examination.

Differential diagnosis CREST syndrome, varicosities, Maffucci syndrome, multiple hemangiomas.

Treatment Supportive.

Hereditary hemorrhgic telangiectasia: multiple lesions on the tongue

CREST Syndrome

CREST syndrome is a clinical variant of scleroderma characterized by a combination of Calcinosis cutis, Reynaud phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia. Telangiectasia may occur on the lips and oral mucosa, and presents as red dots or plaques.

Treatment Surgical excision, or cryotherapy or laser therapy. Some capillary hemangiomas may regress spontaneously.

CREST syndrome: lip telangiectasia

Lupus Erythematosus

Definition Lupus erythematosus is a chronic immunologically mediated disease.

Etiology Autoimmune.

Clinical features Two main forms of the disease are recognized: discoid (DLE) and systemic (SLE). Oral lesions develop in 15–25% of cases in DLE and in 30–45% of cases in SLE, usually in association with skin lesions. The oral lesions are characterized by a well-defined central atrophic red area surrounded by a sharp elevated border of irradiating whitish striae. Telangiectasia, petechiae, edema, erosions, ulcerations, and white hyperkeratotic plaques may be seen.

Laboratory tests Histopathological examination, direct immunofluorescence.

Differential diagnosis Lichen planus, geographic glossitis, speckled leukoplakia, erythroplakia, cicatricial pemphigoid, syphilis.

Treatment Steroids, antimalarials.

Discoid lupus erythematosus: typical lesion on the buccal mucosa

Hemangioma

Definition Hemangioma is a relatively common benign proliferation of blood vessels that primarily develops during childhood.

Etiology Developmental.

Clinical features Two main forms of hemangioma are recognized:capillary and cavernous. The capillary formpresents as a flat red area consisting of numerous small capillaries. Cavernous hemangioma appears as an elevated lesion of a deep red color, and consists of large dilated sinuses filled with blood (Fig. 68). A characteristic sign of hemangioma is that the red color disappears on pressure, and returns when the pressure is released.

Laboratory tests Histopathological examination.

Differential diagnosis Pyogenic granuloma, lymphangioma, traumatic hematoma, Kaposi sarcoma, malignant melanoma.

Capillary hemangioma

Cavernous hemangioma

Gonococcal Stomatitis

Definition Gonococcal stomatitis is a rare manifestation of gonococcal infection. It is a sexually transmitted disease that primarily involves the urinary tract and anorectal area.

Etiology Neisseria gonorhoeae. Gonococcal stomatitis is usually the result of orogenital sex (fellatio, cunnilingus). Accordingly, gonococcal stomatitis is more common in women and homosexual men.

Clinical features The oral manifestations of gonococcal infection appear as atypical fiery erythema and edema with or without superficial ulceration covered with a grayish or yellowish-exudate. A burning, mild pain and sore throat are common symptoms. The pharyngeal area, tonsils, uvula, and soft palate are the most common sites of involvement. Rarely, lesions may develop on the tongue, buccal mucosa, and gingiva. Submandibular and cervical lymph node enlargement may occur. The diagnosis is based on the history and the clinical features, and should be confirmed by identification of the microorganism.

Differential diagnosis Streptococcal stomatitis, herpetic stomatitis, erythematous candidiasis, mechanical trauma, thermal burn.

Treatment Frequently the oral lesions are self-limited. Systemic penicillin or tetracycline are the drugs of choice.

Gonococcal stomatitis, erythema, and erosion on the buccal mucosa

contact allergic stomatitis

Definition Contact allergic stomatitis is a rare acute or chronic allergic reaction.

Etiology Denture base materials, restorative materials, mouthwashes, dentifrices, chewing gums, foods, and other substances may be responsible.

Clinical features Clinically, in the acute form, the affected mucosa presents with diffuse erythema and edema, and occasionally small vesicles and erosions. A burning sensation is a common symptom. In the chronic form, hyperkeratotic white lesions may be seen in addition to erythema.

Laboratory tests Mucosal and skin patch tests.

Differential diagnosis Denture stomatitis, erythematous candidiasis, erythroplakia, leukoplakia, drug reactions.

Treatment Removal of suspected allergens, topical or systemic steroids, antihistamines.

Allergic stomatitis caused by acrylic resin

Linear Gingival Erythema

Definition Linear gingival erythema is a relatively uncommon gingival disorder usually following HIV infection.

Etiology The exact etiology remains unclear. However, a close association of the disorder with Candida infection is common.

Clinical features Characteristically, linear gingival erythema appears as a fiery red band (2–4 mmwide) along the margin of the gingiva and a punctate or diffuse erythema of the attached gingival. Gingival bleeding is rare. The lesion does not respond to plaque control measures or root planing and scaling. The diagnosis is mainly based on the history, the clinical features, and laboratory tests for HIV infection.

Differential diagnosis Plaque-related gingivitis, herpetic gingivitis desquamative gingivitis, granulomatous gingivitis, plasma-cell gingivitis, leukemia.

Treatment High level of oral hygiene and plaque control. Systemic antifungals such as itraconazole or fluconazole are usually useful.

Linear gingival erythema, fiery red band along the margin of the gingiva

Desquamative Gingivitis

Definition Desquamative gingivitis is a clinical descriptive term used for nonspecific gingival manifestation of several chronic mucocutaneous diseases.

Etiology With rare exceptions, an autoimmune mechanism is responsible. Cicatricial pemphigoid and lichen planus are the most common diseases related to desquamative gingivitis. Less frequently, bullous pemphigoid, pemphigus, linear IgA disease, epidermolysis bullosa acquisita, chronic ulcerative stomatitis, discoid lupus erythematosus, and psoriasis may be the underlying disease entity.

Clinical features It presents as erythema and edema of the marginal and attached gingiva. The facial surface is more frequently affected than the lingual gingiva. Spontaneous desquamation of the epithelia, blister formation, and areas of superficial erosions are common. Characteristically, after mild pressure on the affected gingiva, desquamation of the epithelium or hemorrhagic blister formation usually occur. The lesions may be either localized or generalized. Desquamative gingivitis may be the only oral manifestation or may be associated with additional oral lesions of the underlying chronic bullous dermatosis. Women over 40 years of age are more frequently affected. The clinical diagnosis should be confirmed by histopathological and immunological examinations.

Differential diagnosis Necrotizing ulcerative gingivitis, plasma cell gingivitis, plaque related gingivitis, drug reactions, granulomatous gingivitis, oral psoriasis.

Treatment Good oral hygiene, avoidance of any mechanical pressure on the gingiva. Systemic treatment (corticosteroids, immunosuppressants,dapsone) depends on the identification of the underlying disease.

Desquamative gingivitis as a manifestation of cicatricial pemphigoid

Desquamative gingivitis as a manifestation of lichen planus

Granulomatous Gingivitis

Definition Granulomatous gingivitis is a relatively rare, chronic inflammatory disorder with a specific histopathological pattern.

Etiology Foreign body reaction, local bacterial or fungal infection, reactions to food additives and systemic granulomatous disorders (Crohn disease, sarcoidosis, Melkersson–Rosenthal syndrome, Wegener granulomatosis) may be the cause.

Clinical features Granulomatous gingivitis presents as a diffuse erythematous and slightly edematous area of the free and attached gingiva, and of the interdental papillae. The lesions may be localized in one area or multiple areas. Sensitivity and mild pain are common symptoms. The lesions resist conventional local treatment. In cases of systemic granulomatous disease, other areas of the mouth may be involved. The clinical diagnosis should be confirmed by a biopsy and histopathological examination.

Differential diagnosis Plaque-related chronic gingivitis, desquamative gingivitis, linear gingiva erythema, trauma, plasma-cell gingivitis, drug reactions, candidiasis, erythroplakia, non-Hodgkin lymphoma, leukemia, amyloidosis, orofacial granulomatosis.

Treatment Local or systemic corticosteroids is the treatment of choice. In case of reaction to foreign material, conservative surgical excision is suggested.

Granulomatous gingivitis localized

Orofacial granulomatosis, gingival swelling

Plasma-Cell Gingivitis

Definition Plasma-cell gingivitis is a rare and unique gingival disorder, characterized histopathologically by a dense chronic inflammatory infiltration of the lamina propria, mainly of plasma cells.

Etiology Unknown. Reactions to local allergens, chronic infections, and plasma-cell dyscrasias have been considered as possible causes.

Clinical features Clinically, both freeand attached gingiva are bright red and edematous, with a loss ofnormal stippling. Thegingivitismay be localized or widespread, and is frequently accompanied by a burning sensation. Rarely, similar lesions may be seen on the tongue and lips.

Laboratory tests Histopathological and histochemical examination, immunoelectrophoresis.

Differential diagnosis Desquamative gingivitis, psoriasis, candidiasis, soft-tissue plasmacytoma, erythroplakia, granulomatous gingivitis.

Treatment Remove the allergens if possible. Topical or systemic steroids.

Plasma-cell gingivitis

Erythroplakia

Definition Erythroplakia, or Queyrat erythroplasia, is a premalignant lesion frequently occurring on the glans penis, and rarely on the oral mucosa. It is defined as a red, nonspecific patch or plaque that cannot be classified clinically and pathologically under any other disease.

Etiology Unknown.

Clinical features It appears as a usually asymptomatic, fiery red, well demarcated plaque, with a smooth and velvety surface. The red lesions may be associated with white spots or small plaques. The floor of the mouth, retromolar area, soft palate, and tongue are the most common sites of involvement. Erythroplakia occurs more frequently between the ages of 50 and 70 years. Over 91% of erythroplakias histologically demonstrate severe dysplasia, carcinoma in situ, or early invasive squamous-cell carcinoma at the time of diagnosis.

Laboratory tests Histopathological examination.

Differential diagnosis Erythematous candidiasis, lichen planus, discoid lupus erythematous, early squamous-cell carcinoma, local irritation.

Treatment Surgical excision.

Erythroplakia of the buccal mucosa

Erythroplakia of the buccal mucosa.

Erythroplakia of the lateral margin of the tongue

Denture Stomatitis

Definition Denture stomatitis, or denture sore mouth, is a frequent condition in patients who wear dentures continuously for extended times.

Etiology Mechanical irritation fromdentures, Candida albicans, or a tissue response to microorganisms living beneath the dentures.

Clinical features The condition is characterized by diffuse erythema, edema, and sometimes petechiae and white spots that represent accumulations of candidal hyphae, almost always located in the denturebearing area of the maxilla. The condition is usually asymptomatic. The diagnosis is based on clinical criteria.

Differential diagnosis Allergic contact stomatitis due to acrylic.

Treatment Improvement of denture fit, oral hygiene, and topical antimycotics.

Denture stomatitis

Median Rhomboid Glossitis

Definition Median rhomboid glossitis is a rare condition that occurs exclusively on the dorsumof the tongue.

Etiology Presumably developmental. Candida albicans may also be involved.

Clinical features It presents as a well-demarcated erythematous rhomboid area along the midline of the dorsum of the tongue, immediately anterior to the circumvallate papillae. The surface of the lesion may be smooth or lobulated.

Differential diagnosis Candidiasis, lymphangioma, geographic tongue, syphilis, hemangioma, non-Hodgkin lymphoma.

Treatment No treatment is required. 

Median rhomboid glossitis