Indian Journal of Pathology and Microbiology

CASE REPORT
Year
: 2016  |  Volume : 59  |  Issue : 2  |  Page : 216--219

Lichen sclerosus of soft palate: A rare case report


Suyog Tupsakhare, Kishor Patil, Ashok Patil, Shrikant Sonune 
 Department of Oral Pathology and Microbiology, SMBT Dental College and Hospital, Sangamner, Maharashtra, India

Correspondence Address:
Kishor Patil
Department of Oral Pathology and Microbiology, SMBT Dental College and Hospital, Sangamner, Maharashtra
India

Abstract

Lichen sclerosus (LS) is a rare disease affecting the skin and the mucous membrane, and it is chronic inflammatory in nature. It occurs in both males and females, but mainly affects females in the fifth or sixth decade of life. It mainly involves the genital and perianal areas but can affect any part of the body and the involvement of the oral mucosa is exceptionally rare, but sometimes it affects only the oral mucosa. It requires differentiating from other lesions of the oral cavity which looks similar to this lesion. In considering the rarity of the reported cases, the present article reports one more case of LS affecting the soft palate in an edentulous 66 year-old male patient.



How to cite this article:
Tupsakhare S, Patil K, Patil A, Sonune S. Lichen sclerosus of soft palate: A rare case report.Indian J Pathol Microbiol 2016;59:216-219


How to cite this URL:
Tupsakhare S, Patil K, Patil A, Sonune S. Lichen sclerosus of soft palate: A rare case report. Indian J Pathol Microbiol [serial online] 2016 [cited 2020 Oct 29 ];59:216-219
Available from: https://www.ijpmonline.org/text.asp?2016/59/2/216/182044


Full Text

 Introduction



Lichen sclerosus (LS) is a rare type of mucocutaneous disease whose etiology is still not known.[1] It is defined as an uncommon disease of unknown etiology in which characteristic small, white and sclerotic areas occur at any site on the skin, and mucous membrane.[2]

Hallopeau in 1887 has first described the disease as “lichen planus atrophicus.” Later, in 1892, Darier has given the histological description of Lichen sclerosus et atrophicus.[1] Recently, Liver and Schaumburg have described specific histologic criteria.[3] Ravitus, in 1957, has first described the microscopic features of these lesions restricted to oral mucosa only.[4] It is also called as lichen albus, guttate scleroderma, guttate morphea, white spot's disease, and scleroatrophic lichen.[2],[3],[5]

It particularly affects the anogenital area of females in the fifth and sixth decade of life, while extra-genital lesions occur in 15–20% of cases. The oral lesions are extremely rare and affect the oral mucosa alone or associated with genital and/or skin lesions.[6] The lesions are often characterized by shiny macules or papules, which are ivory or porcelain-white in color and generally form plaques. The lesions can be well demarcated and have stiffer consistency.[7] It may cause itching, burning, pain or limitation in mouth opening, but usually are asymptomatic.[8] Atrophy and sclerosis of the skin and mucous membrane are common. LS was initially considered as a variety of lichen planus, but recently it has been regarded as a distinct disease with specific clinical and histopathological features.[1]

The present article reports a case of LS involving only the oral mucosa, which was histopathologically confirmed.

 Case Report



A 67 year-old male patient reported with the complaint of asymptomatic, white patch on palate since 11 years. No history of trauma and increase or decrease in the size of the lesion was reported. Patient had history of tobacco chewing with lime 2–4 times/day for 7–8 years, but the habit was stopped since 6–7 years. The patient had no familial history of similar disease. Clinical examination revealed well-demarcated whitish-gray patch on soft palate, just posterior to the junction of the hard and soft palate on right side of the midline [Figure 1]. It was of approximately 0.8 cm × 0.8 cm in size and slightly elevated from the surrounding mucosa. On palpation, it was non-scrapable, rough in surface structure, non-tender, and stiffer in consistency. Clinical differential diagnosis given was leukoplakia, lichen planus, and candidiasis.{Figure 1}

As the size of then lesion was small, excisional biopsy was performed under local anesthesia and submitted for the histopathological examination[Figure 2]. Microscopic examination of the hematoxylin and eosin stained sections of the excised specimen showed extensive hyperorthokeratosis, atrophic stratified squamous epithelium of variable thickness at places, and hydropic or vacuolar degeneration of the basal cell layer. There was cleft-like space separating the epithelium and the connective tissue [Figure 3]. The subjacent connective tissue showed areas of hyalinization and chronic inflammatory cell infiltration [Figure 4]. Scantiness of the elastic fibers in the connective tissue was revealed by Verhoeff elastic stain (using Van-giesson's as a counter stain) [Figure 5]. Based on the detailed clinical and histopathological examination, final diagnosis of the LS was made. Patient also referred to the dermatological consultation to rule out skin and genital lesions; but there was no evidence of the similar lesion on the skin or genital area. The patient was kept under observation, but there was no evidence of any recurrence or any similar lesion on the skin or genital area after 1 years.{Figure 2}{Figure 3}{Figure 4}{Figure 5}

 Discussion



LS is an unusual mucocutaneous disease which most commonly affects anogenital areas with the genital/extra-genital ratio of 5:1.[3],[6] Its occurrence in the oral mucosa is extremely low, and it affects females 5–10 times more often than males.[3],[5] Most of the extra-genital lesions occurs on the trunk and neck, but any site can be involved.[3] In the oral cavity, it mainly involves buccal mucosa, lips, or gingiva, but can also affect tongue or palate.[1],[7] According to literature, this is the 29th case of oral LS and only oral lesions were present in 17 cases until date. To the best of our knowledge, this is the second case involving the soft palate.

The etiology is unknown, but LS is considered as a multifactorial disease; autoimmune mechanism, genetic factors, trauma, hormonal factors, and various infections have been described.[8],[9] Recent researches indicate that the glycoprotein extracellular matrix protein 1 [ECM1] act as a target antigen and its autoantibodies bring about the pathogenesis. ECM1 play an essential a role in the dermis as it binds to the major heparin sulphate proteoglycan and perlecan; and in the epidermis, it has a role in the keratinocyte differentiation. ECM1, thus helps to regulate interstitial collagen fibril and basement membrane macro-assembly and act as “biological glue.”[9]

Inflammatory infiltrate of CD4+ and CD8+ cells in the connective tissue is responsible for the onset and maintenance of the cutaneous LS. The overall sequence of events leads to microvascular changes, inflammation, altered fibroblast function, and hyaluronic acid accumulation in the upper dermis. All these factors are responsible for the fibrosis of the upper dermis. A cell-mediated autoimmune response may also play an important role in the pathogenesis of the disease.[9]

As the LS clinically resembles to other white lesions, it is mandatory to do histopathological examination for categorization of the lesion appropriately [Table 1].[7] Depending on the appearance of each case, the clinical differential diagnosis includes lichen planus, leukoplakia, localized scleroderma, vitiligo, sub mucous fibrosis, and fibrous scar.[6],[8],[10]{Table 1}

The classical histopathological findings in the cutaneous lesions include hyperkeratosis with follicular plugging, atrophy of the epithelium with vacuolar or hydropic degeneration of the basal cell layer, hyalinization of collagen in the upper dermis and edema. Inflammatory infiltrate are sometimes present. Keratotic plugging is not apparent in mucosal lesions.[11] The subepithelial elastic fibers decrease and results in separation of the epithelial surface at the edematous hyaline area.[6]

Ultrastructural features of LS were studied by Azevedo et al. and observed that the collagen fiber shows broad diameter variation, immature collagen fibers, and loss of cross-striations.[6] Direct immunofluorescence studies have shown IgG, C3, and fibrinogen at the basement membrane level.[1]

The premalignant or malignant transformation can occur in LS patients with genital lesions. In various studies on LS patients of genital lesions, the rate of malignant transformation to squamous cell carcinoma was found to be 3–5%. Till date, no case of malignant transformation in LS patients with oral lesion has been reported. The treatment usually consists of topical and intralesional application of corticosteroids.[6],[8]

 Conclusion



The LS lesions limited to oral cavity are extremely rare. Each case should be assessed separately. Although no cases of malignant transformation was associated with the oral LS, the long-term follow-up is recommended to control the possible appearance of new oral, genital, or skin lesions.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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