Oral lichen sclerosus et atrophicus A case report Waranun Buajeeb, BSc, DDS, MSc (Oral Medicine),a Petcharat Kraivaphan, BSc, DDS, MSc,b Jirapa Punyasingh, BSc, DDS, MSc,c and Penpan Laohapand, BSc, DDS, MDS,a Bangkok, Thailand MAHIDOL UNIVERSITY Lichen sclerosus et atrophicus affecting only the oral mucosa is extremely rare. We report here a case of oral lichen sclerosus et atrophicus presenting as a white, flat lesion involving the right buccal and labial mucosa and vermillion border. The diagnosis was based on histopathologic features. Treatment with intralesional corticosteroid was successful in reducing the size of the lesion and the symptoms of the patient. A free gingival graft was also performed to restore the lost attached gingiva. No recurrence of the lesion was found after a 1-year follow-up period, and no skin or genital lesions developed during the 3 years of treatment. (O ral Surg O ral M ed O ral Pathol O ral Radiol Endod 1999;88:702-6) Lichen sclerosus et atrophicus (LSA) is a rare mucocutaneous disease of unknown etiology. It is characterized by ivory or porcelain-white, round, shiny macules or papules that generally form plaques. Atrophy and sclerosis of the skin and mucous membrane are common. In addition, telangiectasia is frequently found. Genital and perianal involvement are the most prevalent areas of involvement1; however, the condition may be seen in any part of the body. In 1887, Hallopeau2 first described the disease as “lichen planus atrophicus.” Later, in 1892, Darier3 reported the histopathologic features of a similar lesion called “lichen planus sclerosus.” Although LSA was initially considered to be a variety of lichen planus,2,3 it has recently been regarded as a distinctive disease with specific clinical and histopathologic features.4,5 LSA involving the oral mucosa is extremely rare. It can affect the buccal and labial mucosa, gingiva, palate, or vermillion border, with or without skin or genital lesions. Only 19 cases have been reported in the literature.6-14 Of these, 5 cases were histologically proven oral LSA without any skin or genital lesions after a follow-up period (Table I). The purpose of this report is to document a new case of LSA involving only the oral mucosa and to discuss the treatment of this disorder. CASE REPO RT A 22-year-old Thai woman was referred by her dentist to the Department of Oral Medicine, Faculty of Dentistry, Mahidol aAssociate Professor, Department of Oral Medicine, Faculty of Dentistry. bAssociate Professor, Department of Pharmacy, Faculty of Dentistry. cAssociate Professor, Department of Oral Pathology, Faculty of Dentistry. Received for publication Mar 1, 1999; returned for revision Apr 8, 1999; accepted for publication May 25, 1999. Copyright © 1999 by Mosby, Inc. 1079-2104/99/$8.00 + 0 7/14/100426 702 Fig 1. Clinical appearance shows white lesion on buccal mucosa and mucobuccal fold. University, Bangkok, Thailand, for examination and treatment of a white lesion on her oral mucosa. Her chief complaints were slight tightness on opening at the right buccal mucosa and soreness of the mandibular right gingiva on tooth brushing. Her medical history disclosed that she had had asthma since childhood; the last attack was 6 years previously. The patient was otherwise well, and she was taking no medication. She did not drink alcohol, smoke tobacco, or chew areca nut. No history of trauma or surgery in the area of the mandibular right buccal mucosa and gingiva was disclosed. Clinical examination revealed a white macular lesion on the patient’s right buccal mucosa and mucobuccal fold from the distal aspect of the mandibular right second molar to the labial aspect of the mandibular right canine, extending anteriorly onto the labial mucosa and vermillion border of the lower lip (Fig 1). The lesion was approximately 2 × 7 cm in size, and on palpation it was firmer than the surrounding areas. The patient’s oral hygiene was good; she had mild generalized gingivitis except for the mandibular right quadrant. Gingival recession and moderate inflammation were noted at the buccal and lingual aspects of the mandibular right second molar to canine. No Buajeeb et al 703 ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Volume 88, Number 6 Fig 2. Photomicrograph shows hyperparakeratosis, hydropic degeneration of basal cells, subepithelial hyalinization, and underlying inflammatory cell infiltrate (hematoxylin-eosin, original magnification ×100). Table I. Histologically verified reported cases of lichen sclerosus et atrophicus involving oral mucosa only Author(s) Year of publication Patient’s sex Patient’s age (y) Oral site(s) Ravits7 Macleod and Soames12 Schulten et al13 1957 1991 1993 Brown et al14 1997 M F F M M 24 57 59 12 44 Buccal mucosa and gingiva Tongue and palate Labial mucosa, commissure and tongue Labial mucosa Soft palate midline attached gingiva was found at the buccal side of these teeth; this caused movement of the free gingiva during mucosal retraction. There was no evidence of genital or skin lesions. Radiographic examination showed normal findings except for a thickening of the periodontal ligament space and lamina dura at the mandibular right first molar. Laboratory investigations revealed a hemoglobin level of 12.8 g/dL (12-16 g/dL), a white blood cell count of 7.6 × 109/L (5.0-10.0×109/L), and a normal platelet count. Liver function was within normal limits. The patient’s antinuclear antibody titers were negative, with normal levels of IgG, IgA, and IgM. H istopathologic findings An incisional biopsy of the lesion was performed on the labial mucosa with the patient under local anesthesia. Examination of sections stained with hematoxylin and eosin revealed a mucosal strip with minor salivary glands. The epithelium showed atrophy and focal areas of hyperparakeratosis. Hydropic degeneration of the basal cells and intracellular edema of the spinous layers were noted occasionally. The underlying connective tissue revealed hyalinization of a band of the connective tissue with inflammatory cells and vessels (Figs 2 and 3). Telangiectasia of the vessels was also noted. Immunofluorescent staining of the section was negative for immunoglobulins G, M, and A as well as C3 and fibrinogen. A section stained by Verhoeff’s method showed scantiness of elastic fibers in the superficial connective tissue. It was concluded that the histopathologic features were consistent with a diagnosis of LSA. Treatment Scaling, root planing, and oral hygiene procedures were performed regularly; the patient herself could not clean her mandibular right teeth properly because of soreness during tooth brushing. The patient asked for relief of tightening at the right buccal mucosa. Topical triamcinolone acetonide 0.1% (Kenalog in Orabase) was prescribed; it was applied 4 times daily for approximately 2 months, without any evidence of regression of the lesion. Therefore, methylprednisolone acetate 40 mg/mL (Depo-Medrol 40) was used, 0.1 mL per 1 cm2 being injected on divided areas. The lesion was injected in 3 or 4 areas each time; this made for a series of 5 treatments 704 Buajeeb et al ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY December 1999 Fig 4. After free soft tissue grafting, attached gingiva has been gained at buccal aspect of teeth in right mandibular quadrant. Fig 3. Photomicrograph shows hyperparakeratosis, intracellular edema of spinous cell layer, and hyalinization of collagen in upper lamina propria (hematoxylin-eosin, original magnification ×200). with 1-month intervals. Subsequent examinations showed regression in the size of the lesion on the buccal mucosa and disappearance of the white lesion on the labial mucosa. On palpation, the lesion was softer than it had been previously. The patient felt less tightening at the right buccal mucosa; however, soreness and inflammation of the mandibular right gingiva still existed. Therefore, a free soft tissue graft (from the palatal mucosa) was performed at the buccal aspect of the mandibular right second molar to canine to restore the lost attached gingiva. After grafting, a significant increase in attached gingiva was achieved (Fig 4) and the patient could brush her teeth properly without soreness of the area. D ISCU SSIO N According to the reports in the literature, the prevalence of LSA affecting the oral mucosa alone is extremely rare. Five cases with histologically verified oral LSA have been reported.7,12-14 No skin or genital lesions developed after the follow-up period in any of the reported cases or in our case. This suggests that oral LSA may appear without accompanying skin or genital lesions. Oral LSA has been described on labial and buccal mucosa, lip, gingiva, palate, tongue, and anterior tonsillar pillar. The lesions manifest as white macules or plaques,6-9,13,14 with reticular striations6,10 and superficial ulceration.11,12 Clinically, it may be difficult to distinguish oral LSA from other oral white lesions, especially lichen planus,6,9,10 leukoplakia,15 submucous fibrosis,10 and localized scleroderma.15 Oral lichen planus usually presents as white reticular striations. A minority of patients with oral lichen planus develop dense, white, homogeneous plaques.16 Oral leukoplakia shows a variety of appearances, ranging from fine white flecks to dense thick plaques.16 It may be seen in smokers and users of smokeless tobacco. In the present case, the firm white macule was difficult to differentiate clinically from submucous fibrosis and localized scleroderma. However, the patient had no history of chewing areca nut and the lesion was localized; submucous fibrosis could therefore be excluded clinically. LSA is asymptomatic, an exception being the case reported by Siar and Ng,10 in which the symptoms probably resulted from atrophy of the lesion. Except for slight tightening and soreness at the adjacent buccal gingiva during tooth brushing, our patient was asymptomatic. Both of her symptoms could be explained by sclerosis of the lesion. The lack of attached gingiva and the inflamed marginal tissue also caused the soreness. The histologic features of cutaneous LSA are characteristic, including follicular plugging, atrophy of the epidermis with vacuolar degeneration of the basal layer, edema, homogenization of the collagen and scantiness or loss of elastic fibers in the upper dermis, and an inflammatory infiltrate in the mid dermis.5 The histologic features of oral LSA are quite similar to those of LSA of the skin. De Araujo et al9 have concluded that the characteristic features of oral LSA are pronounced edema and homogenization of the Buajeeb et al 705 ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Volume 88, Number 6 Table II. Comparison of histologic features of oral mucosal lesions with similar clinical appearances Oral lesions Epithelium Basal cell liquefaction LSA Lichen planus Leukoplakia Submucous fibrosis Scleroderma Hyperkeratosis to atrophy Hyperkeratosis to atrophy Hyperkeratosis to atrophic parakeratosis Atrophy Atrophy +/– + – – – collagen fibers within the lamina propria, together with hydropic degeneration of the basal cells. The density of the mononuclear infiltrate is greater in early lesions than in longstanding lesions.17 In addition, the younger the lesion, the more superficial the infiltrate.5 It is sometimes difficult to differentiate oral LSA from oral lichen planus, oral submucous fibrosis, and oral scleroderma by means of histologic features. However, the distinct band of lymphocytes at the epithelial-connective tissue junction that is usually found in lichen planus is not evident in LSA.18 The distinction from oral submucous fibrosis is more difficult. However, pyknotic changes in the nuclei of the basal cell layer,19 epithelial atypia,20 and obliteration or narrowing of the blood vessels21 are not observed in LSA. Unlike scleroderma, in which the dominant pathologic process involves deep dermis, LSA involves only the superficial dermis.17 Furthermore, scantiness or loss of elastic fibers, characteristic of LSA, is not found in scleroderma.22 Collagen biosynthesis is increased in scleroderma but decreased in LSA.23 Although our case clinically resembled oral submucous fibrosis, the histopathologic features were used to differentiate oral LSA from oral submucous fibrosis; furthermore, telangiectasia and the scantiness of elastic fibers localized in the upper lamina propria were used to differentiate oral LSA from scleroderma. Therefore, histologic findings were critical in making a diagnosis of oral LSA. With respect to some histologic features, oral LSA is compared with other oral white lesions in Table II. The etiology of LSA is unknown, but many investigators believe the condition to be autoimmune in nature. It was found to be strongly correlated with autoimmune diseases such as thyroiditis, insulin-dependent diabetes mellitus, and vitiligo.24 Direct immunofluorescent studies may reveal immunoglobulin G, C3, and fibrinogen at the basement membrane level.25,26 In our case, negative immunofluorescent staining was reported. In addition, the patient had no autoimmune diseases. The histopathologic findings were consistent with the diagnosis of LSA. Other possible causes, such Band of lymphocytes at Scantiness epithelium connective or loss tissue junction of elastic fibers – + – – – + – – – – Obliteration or narrowing of blood vessels – – – + + as traumatic, genetic, and hormonal factors, were not found to be related in this case. Treatment of oral LSA is usually unnecessary because of its asymptomatic nature. Topical application of corticosteroids has been reported, the outcome being variable.10,12 In our patient, the efficacy of intralesional corticosteroids was demonstrated. Gingival recession resulting from oral LSA affecting the gingiva, which was never before reported, is probably caused by sclerosis of the adjacent buccal mucosa. We succeeded in grafting the attached gingiva on the buccal aspect of the mandibular right second molar to canine, which helped to restore the gingival health because the patient could then clean her teeth properly without soreness. There was no evidence of recurrence of the lesion after 3 months of follow-up. No skin or genital lesions developed during 3 years of follow-up. REFEREN CES 1. Wallace HJ. Lichen sclerosus et atrophicus. Trans St John’s Hosp Dermatol Soc 1971;57:9-30. 2. Hallopeau H. Du lichen plan et particulierement de sa forme atrophique: lichen plan sclereux. Ann Dermatol Syph 1887;8:7901. 3. Darier J. Lichen plan sclereux. Ann Dermatol Syph 1892;23:8337. 4. Ridley CM. Lichen sclerosus. Dermatol Clin 1992;10:309-18. 5. Lever WF, Schaumburg-Lever G. Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott; 1990. p. 308-12. 6. Miller RA. Lichen sclerosus et atrophicus with oral involvement. Arch Dermatol 1957;76:43-55. 7. Ravits HG. Lichen sclerosus et atrophicus of the mouth. Arch Dermatol 1957;76:56-8. 8. Kaminsky CA, Kaminsky AR, Abulafia J. Liquen escleroso y atrófico de piel y mucosa bucal. Medicina Cutanea Ibero-LatinoAmericana 1974;2:87-92. 9. De Araujo CV, Orsini SC, Marcucci G, De Araujo NS. Lichen sclerosus et atrophicus. Oral Surg Oral Med Oral Pathol 1985;60:6557. 10. Siar CH, Ng KH. Oral lichen sclerosus et atrophicus: report of case. J Oral Med 1985;40:148-50. 11. Dalziel K, Reynolds AJ, Holt PJA. Lichen sclerosus et atrophicus with ocular and maxillary complications. Br J Dermatol 1987;116:735-7. 12. Macleod RI, Soames JV. Lichen sclerosus et atrophicus of the oral mucosa. Br J Oral Maxillofac Surg 1991;29:64-5. 13. Schulten E, Starink TM, Van Der Waal I. Lichen sclerosus et atrophicus involving the oral mucosa: report of two cases. J Oral Pathol Med 1993;22:374-7. 14. Brown AR, Dunlap CL, Bussard DA, Lask JT. Lichen sclerosus 706 Buajeeb et al 15. 16. 17. 18. 19. 20. 21. 22. ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY December 1999 et atrophicus of the oral cavity: report of two cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;84:165-70. Bengle W, Veltman G, Loevy HT, Taschini P. Differential diagnosis of diseases of the oral mucosa. Chicago: Quintessence Publishing; 1989. p. 129-31. Cawson RA, Binnie WH, Eveson JW. Color atlas of oral diseases: clinical and pathologic correlations. 2nd ed. London: Wolfe; 1994. p. 12.17-12.18. Ackerman AB. Histologic diagnosis of inflammatory skin diseases. Philadelphia: Lea & Febiger; 1978. p. 760-4. Rook AJ, Wilkinson DS, Ebling FJ. Textbook of dermatology. 5th ed. Oxford: Blackwell Scientific Publications; 1979. p. 1229. Mani NJ, Singh B. Studies on oral submucous fibrosis, III: epithelial changes. Oral Surg Oral Med Oral Pathol 1976;41:203-14. Pindborg JJ. Oral submucous fibrosis as a precancerous condition. J Dent Res Suppl 1966;45:546-53. Shafer WG, Hine MK, Levy BM. A textbook of oral pathology. 4th ed. Philadelphia: WB Saunders Co; 1983. p. 109-10. Winer LH. Elastic fibers in unusual dermatoses. Arch Dermatol 1955;71:338-48. 23. Panizzon R, Vuorio T, Bruckner-Tuderman L. Collagen biosynthesis and type I and type III procollagen mRNA in lichen sclerosus et atrophicus. Arch Dermatol Res 1990;2:283-6. 24. Meyrick RH, Ridley CM, McGibbon DH, Black MM. Lichen sclerosus et atrophicus and autoimmunity: a study of 350 women. Br J Dermatol 1988;118:41-6. 25. Dickie RJ, Horne CH, Sutherland HW, Bewsher PD, Stankler L. Direct evidence of localized immunological damage in vulvar lichen sclerosus et atrophicus. J Clin Pathol 1982;35:1395-7. 26. Bushkell LL, Friedrich EG, Jordan RE. An appraisal of routine direct immunofluorescence in vulvar disorders. Acta Derm Venereol (Stockh) 1981;61:157-61. Reprint requests: Waranun Buajeeb, BSc, DDS, MSc Department of Oral Medicine, Faculty of Dentistry, Mahidol University 6 Yothi Street, Rachathavee, Payathai Bangkok, Thailand 10400 BO U N D VO LU M ES AVAILABLE TO SU BSCRIBERS Bound volumes of Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics are available to subscribers (only) from the Publisher, at a cost of $92.00 for domestic, $112.35 for Canadian, and $105.00 for international, for Vol 87 (January-June) and Vol 88 (July-December). Shipping charges are included. Each bound volume contains a subject and author index, and all advertising is removed. Copies are shipped within 60 days after publication of the last issue in the volume. The binding is durable buckram with the journal name, volume number, and year stamped in gold on the spine. 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