Peer Reviewed

Photo Essay

An Atlas of Lingual Lesions, Part 4

Alexander K. C. Leung, MD
Clinical Professor of Pediatrics, University of Calgary; Pediatric Consultant, Alberta Children’s Hospital, Calgary, Alberta, Canada

Benjamin Barankin, MD
Dermatologist, Medical Director, and Founder, Toronto Dermatology Centre, Toronto, Ontario, Canada

Kin Fon Leong, MD
Pediatric Dermatologist, Pediatric Institute, Kuala Lumpur General Hospital, Kuala Lumpur, Malaysia

Alex H. Wong, MD
Clinical Assistant Professor of Family Medicine, University of Calgary, Calgary, Alberta, Canada

CITATION:
Leung AKC, Barankin B, Leong KF, Wong AH. An atlas of lingual lesions, part 4. Consultant. 2019;59(8):242-245.

EDITOR’S NOTE: This article is part 4 of a 5-part series of Photo Essays describing and differentiating conditions affecting the tongue and related structures in the oral cavity. Part 1 was published in the May 2019 issue (https://www.consultant360.com/article/consultant360/atlas-lingual-lesions-part-1), part 2 was published in the June 2019 issue (https://www.consultant360.com/article/consultant360/atlas-lingual-lesions-part-2), and part 3 was published in the July 2019 issue (https://www.consultant360.com/article/consultant360/atlas-lingual-lesions-part-3). Part 5 will be published in an upcoming issue of Consultant.

 

Lichen Planus

Lichen planus is an inflammatory dermatosis of unknown origin that typically affects the skin, mucous membranes, and nails. One or several areas can be involved, either concomitantly or sequentially.1

Cutaneous lichen planus is the most common presentation characterized by 6 p’s: planar (flat-topped), purple (violaceous), polygonal, pruritic, and papules/plaques that affect the skin (Figure 1).1,2 The lesions of lichen planus are often superimposed by lacy, reticular, white lines known as Wickham striae, best seen in the buccal mucosa or after skin lesions are swiped with an alcohol wipe.1,2 Sites of predilection include the flexor aspects of the wrists and ankles, dorsa of hands, trunk, shins, and glans penis.3 The distribution is often symmetric. As with psoriasis, the Koebner phenomenon is particularly characteristic.2

Fig 1
Figure 1.

There are 3 main forms of oral lichen planus: reticular, erosive/ulcerative, and atrophic.2,4 By far, the most common site is the buccal mucosa, followed by the tongue, gingiva, and vestibule.4 The reticular form is most common and typically presents as diffuse, bilateral, asymptomatic papules or plaques interlaced with Wickham striae on the oral mucosa.4,5 The erosive form presents as ulceration, erythema, and keratotic areas, as is illustrated in Figure 2. Patients with erosive lesions may report pain or a burning sensation in the mouth, exacerbated by eating acidic or spicy foods. The atrophic form presents as a red, diffuse lesion with mucosal atrophy. Other forms include plaque-like, hypertrophic, papular, and bullous.6 Sites of predilection are the buccal mucosa, tongue, and gingiva.7-9 Involvement of the palate, lips, and floor of the mouth is rare.9 The diagnosis is mainly a clinical one. A biopsy should be considered if the diagnosis is in doubt.

Fig 2
Figure 2.

The prevalence of oral lichen planus has been estimated to be 0.2% to 2.2% of the population.8-10 The female to male ratio is 1.5 to 3:1.11 The typical age of onset is between 30 and 60 years of age.9

Although the exact etiology is not known, an immune-mediated pathogenesis has been recognized.7 The overrepresentation of certain HLA haplotypes (eg, HLA-A3, HLA-A5, HLA-B8, HLA-Bw35, and HLA-DR1) suggests that genetic factors may be operative.12 There is an association between oral lichen planus and hepatitis C virus infection.13

The erosive form of oral lichen planus can be quite painful and may interfere with speech, chewing, gustatory function, and swallowing and even result in weight loss.4,5,11,14 Whereas cutaneous lichen planus is self-limited, oral lichen planus tends to run a chronic, persistent course characterized by remissions and relapses.8-10 Complete remission is rare.7,10 A 2018 systematic review of 21 studies enrolling a total of 6559 patients showed that the malignant transformation rate to squamous cell carcinoma with oral-erosive lichen planus was 1.37%.15 Female gender, involvement of the tongue, and erosive type of the lesion increased the malignant transformation rate.15

Because oral lichen planus is an immunologically related disease, medium- to high-potency topical corticosteroids are the treatment of choice.11,16 Other treatment options include topical calcineurin inhibitors, and systemic cyclosporine, mycophenolate mofetil, acitretin, or methotrexate.6,11,17 Oral corticosteroids may be considered in recalcitrant cases.11

REFERENCES:

  1. Leung AKC, Barankin B. Lichen planus. Consultant. 2014;54(2):137-138.
  2. Usatine RP, Tinitigan M. Diagnosis and treatment of lichen planus. Am Fam Physician. 2011;84(1):53-60.
  3. Lehman JS, Tollefson MM, Gibson LE. Lichen planus. Int J Dermatol. 2009;​48(7):682-694.
  4. Hargitai IA. Painful oral lesions. Dent Clin North Am. 2018;62(4):597-609.
  5. Au J, Patel D, Campbell JH. Oral lichen planus. Oral Maxillofacial Surg Clin North Am. 2013;25(1):93-100.
  6. Gupta A, Sardana K, Gautam RK. Looking beyond the cyclosporine “swish and spit” technique in a recalcitrant case of erosive lichen planus involving the tongue. Case Rep Dermatol. 2017;9(3):177-183.
  7. Barbosa NG, Silveira ÉJD, Lima ENA, Oliveira PT, Soares MSM, de Medeiros AMC. Factors associated with clinical characteristics and symptoms in a case series of oral lichen planus. Int J Dermatol. 2015;54(1):e1-e6.
  8. López-Jornet P, Camacho-Alonso F. Tongue involvement in patients with lichen planus. A retrospective study. J Eur Acad Dermatol Venereol. 2009;​23(2):204-205.
  9. Werneck JT, Costa TdO, Stibich CA, Leite CA, Dias EP, Silva Junior A. Oral lichen planus: study of 21 cases. An Bras Dermatol. 2015;90(3):321-326.
  10. Padmini C, Bai KY, Chaitanya V, Reddy MS. Ulcerative lichen planus in childhood. Case Rep Dent. 2013;2013:874895.
  11. Park H-K, Hurwitz S, Woo S-B. Oral lichen planus: REU scoring system correlates with pain. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;114(1):​75-82.
  12. Kanwar AJ, De D. Lichen planus in childhood: report of 100 cases. Clin Exp Dermatol. 2009;35(3):257-262.
  13. Lauritano D, Arrica M, Lucchese A, et al. Oral lichen planus clinical characteristics in Italian patients: a retrospective analysis. Head Face Med. 2016;​12:18.
  14. Suter VGA, Negoias S, Friedrich H, Landis BN, Caversaccio M-D, Bornstein MM. Gustatory function and taste perception in patients with oral lichen planus and tongue involvement. Clin Oral Investig. 2017;21(3):957-964.
  15. Richards D. Malignant transformation rates in oral lichen planus. Evid Based Dent. 2018;19(4):122.
  16. Chiang C-P, Chang JY-F, Wang Y-P, Wu Y-H, Lu S-Y, Sun A. Oral lichen planus—differential diagnoses, serum autoantibodies, hematinic deficiencies, and management. J Formos Med Assoc. 2018;117(9):756-765.
  17. Mirza S, Rehman N, Alrahlah A, Alamri WR, Vohra F. Efficacy of photodynamic therapy or low level laser therapy against steroid therapy in the treatment of erosive-atrophic oral lichen planus. Photodiagnosis Photodyn Ther. 2018;21:404-408.

NEXT: Sarcoidosis

Sarcoidosis

Sarcoidosis is a multisystem granulomatous disease characterized by the formation of noncaseating (non-necrotizing) epithelial cell granulomas in various tissues and organs.1,2 The condition may be asymptomatic; however, it may also present with nonspecific constitutional symptoms (eg, fever, malaise, fatigue, weight loss) or with symptoms related to specific organ involvement.3,4

Approximately 90% of patients with sarcoidosis present with pulmonary manifestations (eg, dry cough, shortness of breath, chest pain), approximately 25% with cutaneous manifestations (eg, maculopapular eruption; erythematous scaling plaques; erythema nodosum; hypopigmentation or hyperpigmentation; scar or keloid), and approximately 13% with head and neck manifestations.5-8 In the head and neck region, the most commonly affected structures are the salivary glands and the cervical lymph nodes.1 The former may present as enlargement of the salivary glands and xerostomia and the latter as cervical lymphadenopathy (usually bilateral).2 Oral involvement is relatively rare and most often affects the buccal mucosa (30%), followed by the gingiva (20%), the tongue (16%), the lips (16%), and the palate (9%).9 Typically, oral sarcoidosis presents as one or more nontender firm swellings or nodules with normal overlying mucous membranes, as is illustrated in the Figure.7,9 At times, oral sarcoidosis presents as painless ulcers, gingivitis, gingival hyperplasia, or gingival recession.3,6

sarcoidosis

In the United States, the prevalence rate of sarcoidosis is 10 to 14 cases per 100,000 in the white population and 35.5 to 64 cases per 100,000 in the African American population.1,4,10 The age of onset peaks between 20 and 29 years and again between 45 and 65 years.2,3 The female to male ratio is 1.5 to 1.7,11

The exact etiology of sarcoidosis is not known. Presumably, the condition results from an exaggerated immune response to unknown environmental antigens in genetically susceptible individuals.12 The genetic predisposition is suggested by familial clustering, ethnic variations of disease prevalence, and increased concordance in monozygotic twins.1,5 Sarcoidosis is familial in 3.6% to 9.6% of cases, suggesting an autosomal recessive mode of inheritance with incomplete penetrance.12 Suffice to say, the majority of cases are sporadic.13

The diagnosis of sarcoidosis is mainly based on characteristic clinical features, typical radiologic features (bilateral hilar lymphadenopathy, pulmonary infiltrates), and histologic findings of noncaseating epithelial cell granulomas replete with Langerhans giant cells and a rim of lymphocytes.2,3,14 Supportive laboratory findings include elevation of acute-phase reactants such as erythrocyte sedimentation rate and C-reactive protein level, anemia, lymphocytopenia, elevated liver enzyme levels, and an increased serum angiotensin-converting enzyme level.4,10

Treatment depends on the extent and severity of the disease. No treatment is necessary for oral sarcoidosis apart from watchful observation in those with asymptomatic, mild, and focal disease, since spontaneous resolution is common.5,11 Topical and intralesional corticosteroids may be considered for limited or localized mucocutaneous involvement.5 Surgical excision is the treatment of choice for isolated, accessible, circumscribed, nodular lesions that are uncomfortable.11,13 For widespread and progressive lesions, systemic corticosteroids are the treatment of choice.13 For those who do not respond favorably to systemic corticosteroids, adjunct treatment with steroid-sparing drugs such as cyclosporine, methotrexate, cyclophosphamide, azathioprine, chlorambucil, pentoxifylline, infliximab, adalimumab, chloroquine, and hydroxychloroquine may be considered.3,10,12

REFERENCES:

  1. Al-Azri AR, Logan RM, Goss AN. Oral lesion as the first clinical presentation in sarcoidosis: a case report. Oman Med J. 2012;27(3):243-245.
  2. Radochová V, Radocha J, Laco J, Slezák R. Oral manifestation of sarcoidosis: a case report and review of the literature. J Indian Soc Periodontol. 2016;20(6):627-629.
  3. Bowers L, Brennan M. Oral complications of multiorgan disorders. Atlas Oral Maxillofac Surg Clin North Am. 2017;25(2):187-195.
  4. Dastoori M, Fedele S, Leao JC, Porter SR. Sarcoidosis—a clinically orientated review. J Oral Pathol Med. 2013;42(4):281-289.
  5. Alawi F. An update on granulomatous diseases of the oral tissues. Dent Clin North Am. 2013;57(4):657-671.
  6. Gill I, Siddiqi J. An oral lesion as the primary clinical manifestation of sarcoidosis. Ann R Coll Surg Engl. 2017;99(5):e135-e136.
  7. Gupta S, Tripathi AK, Kumar V, Saimbi CS. Sarcoidosis: oral and extra-oral manifestation. J Indian Soc Periodontol. 2015;19(5):582-585.
  8. Leung AKC, Leong KF, Lam JM. Erythema nodosum. World J Pediatr. 2018;​14(6):548-554.
  9. Bouaziz A, Le Scanff J, Chapelon-Abric C, et al; Groupe Sarcoïdose Francophone. Oral involvement in sarcoidosis: report of 12 cases. QJM. 2012;​105(8):755-767.
  10. Suresh L, Radfar L. Oral sarcoidosis: a review of literature. Oral Dis. 2005;​11(3):138-145.
  11. Marcoval J, Mañá J. Specific (granulomatous) oral lesions of sarcoidosis: report of two cases. Med Oral Patol Oral Cir Bucal. 2010;15(3):e456-e458.
  12. Sankar V, Noujeim M. Oral manifestations of autoimmune and connective tissue disorders. Atlas Oral Maxillofac Surg Clin North Am. 2017;25(2):113-126.
  13. Motswaledi MH, Khammissa RA, Jadwat Y, Lemmer J, Feller L. Oral sarcoidosis: a case report and review of the literature. Aust Dent J. 2014;59(3):389-394.
  14. Tripathi P, Aggarwal J, Chopra D, Bagga S, Sethi K. Sarcoidosis presenting as isolated gingival enlargement: a rare case entity. J Clin Diagn Res. 2014;​8(11):ZD25-ZD26.

NEXT: Hemangioma

Hemangioma

Hemangiomas are benign vascular tumors due to proliferation of endothelial cells.1 Characteristically, hemangiomas are not clinically apparent at birth.2 They usually appear in the first few weeks of life as areas of pallor, followed by telangiectatic patches.2 These lesions are characterized by a distinctive life cycle in which proliferation is generally limited to the first year of life, at which time the growth rate slows to parallel the growth of the child, followed by a variable involution phase.2-5

Typically, hemangiomas are asymptomatic.2 Superficial lesions are bright red, protuberant, and sharply demarcated and are often referred to as “strawberry hemangiomas” or “capillary hemangiomas.”2 Deep lesions are bluish and dome-shaped and are noted later than superficial hemangiomas.2 They reach their maximum size between 1 and 2 years of age.2 Deep hemangiomas consists of deep, irregular, large, thin-walled cavernous vessels and sinusoids lined by epithelial cells and separated by a scanty connective tissue stroma.1,3 They feel like a “bag of worms” and are compressible; they are often referred to as “cavernous hemangiomas.”6 Approximately 60% of hemangiomas are superficial, 15% are deep, and 25% are mixed superficial and deep.6 Mixed hemangiomas (both superficial and deep) may show characteristic features of both, often presenting with a red plaque overlying a bluish nodule.2

Hemangioma

Although hemangiomas can appear anywhere on the skin, internal organs, or mucous membranes, 60% to 70% of cases occur in the head and neck region.3 However, occurrence within the oral cavity, in particular the tongue, is exceeding rare.3-7 Typically, oral hemangiomas present at an older age than lesions elsewhere, usually between the second and fourth decade.8 Most lingual hemangiomas are cavernous or mixed in nature and often present as a smooth, lobulated mass or as macroglossia (Figure).5,7 They are dark red-blue or deep red in color and soft to firm in consistency.7,8

A lingual hemangioma must be differentiated from a venolymphatic malformation, arteriovenous malformation, and lymphangioma. In the case of the patient shown in the Figure, color Doppler ultrasonography of the tongue showed a huge echogenic lesion with intermittent color picking, suggestive of a vascular lesion, and small cystic areas and increased vascularity, confirming the diagnosis of hemangioma causing macroglossia.

In the white population, hemangioma affects approximately 1.1% to 2.6% of newborn infants and 10% to 12% of children by the first year of life.9 The female to male ratio is approximately 3 to 1.4

Hemangiomas arise from endothelial stem cells that later proliferate by vasculogenesis, with further angiogenesis.2 Hypoxia and estrogen are important stimuli and have a synergistic effect on angiogenesis.10 The genes encoding vascular endothelial growth factor, indoleamine 2,3-dioxygenase, insulinlike growth factor 2, angiopoietin 1, angiopoietin 2, basic fibroblast growth factor, Cip1-interacting zinc finger protein 1, and tyrosine-protein kinase receptor (Tie2) are believed to play a significant role in the pathogenesis of hemangiomas.3,9,10

Complications include cosmetic disfigurement, recurrent trauma due to biting of the tongue, hemorrhage, ulceration, infection in an ulcerated lesion, chewing/swallowing/feeding difficulties, speech impairment, and airway obstruction.1,3,11-13 In general, the risk of complications is closely related to the size of the lesion.

Treatment should be individualized. Most lingual hemangiomas, especially small ones, may not require intervention except reassurance and watchful observation.1,3 Indications for active intervention include severe or recurrent hemorrhage, life- or function-threatening complications, pedunculated hemangiomas, and significant disfigurement.2 When treatment is necessary, surgical excision is the treatment of choice when feasible. Other treatment options include intralesional corticosteroids, oral β-blockers, sclerotherapy, laser surgery, laser photocoagulation, electrocautery, cryotherapy, immunomodulatory therapy with interferon alfa-2a, embolization, radiofrequency tissue ablation, and ligation.1,3,4,14,15

REFERENCES:

  1. Portaro S, Naro A, Guarneri C, Di Toro G, Manuli A, Calabrò RS. Hemangiomas of the tongue and the oral cavity in a myotonic dystrophy type 1 patient: a case report. Medicine (Baltimore). 2018;97(48):e13448.
  2. Leung AKC, Barankin B, Hon KL. Infantile hemangioma. Pediatr Neonatal Nurs Open J. 2014;1(1):1-6.
  3. Kamala KA, Ashok L, Sujatha GP. Cavernous hemangioma of the tongue: a rare case report. Contemp Clin Dent. 2014;5(1):95-98.
  4. V P, Puppala N, Deshmukh SN, Jagadesh B, Anuradha S. Cavernous hemangioma of tongue: management of two cases. J Clin Diagn Res. 2014;​8(10):ZD15-ZD17.
  5. Shrestha AL, Paudel SB. Lingual cavernous hemangioma in a Nepalese boy—‘a difficult associate!!!’ J Surg Case Rep. 2018;2018(10):rjy283.
  6. Atherton DJ. Infantile hemangiomas. Early Hum Dev. 2006;82(12):789-795.
  7. Kripal K, Rajan S, Ropak B, Jayanti I. Cavernous hemangioma of the tongue. Case Rep Dent. 2013;2013:898692.
  8. Tasker LJ, Geoghegan J. Giant cavernous haemangioma of the tongue. Anaesthesia. 2005;60(10):1043.
  9. Lo K, Mihm M, Fay A. Current theories on the pathogenesis of infantile hemangioma. Semin Ophthalmol. 2009;24(3):172-177.
  10. Chen TS, Eichenfield LF, Friedlander SF. Infantile hemangioma: an update on pathogenesis and therapy. Pediatrics. 2013;131(1):99-108.
  11. Wang Y, Li X, Zhang J, et al. CIZ1 expression is upregulated in hemangioma of the tongue [published online November 19, 2018. Pathol Oncol Res. doi:10.1007/s12253-018-0495-4.
  12. 12. Gallarreta FW, Pieroni KA, Mantovani CP, Silva FW, Nelson-Filho P, de Queiroz AM. Oral changes stemming from hemangioma of the tongue. Pediatr Dent. 2013;35(2):E75-E78.
  13. Rudingwa P, Sundararajan V, Vasudevan A, Pannerselvam S. An innovative airway management technique in an infant with tongue hemangioma. Paediatr Anaesth. 2019;29(4):389-390.
  14. Atkins JH, Mandel JE, Mirza N. Laser ablation of a large tongue hemangioma with remifentanil analgosedation in the ORL endoscopy suite. ORL J Otorhinolaryngol Relat Spec. 2011;73(3):166-169.
  15. Nip SYA, Hon KL, Leung WKA, Leung AKC, Choi PCL. Neonatal abdominal hemangiomatosis: propranolol beyond infantile hemangioma. Case Rep Pediatr. 2016;2016:9803975.