Lichen Planus with extensive cutaneous and oral manifestations--a case report and review of literature.
Lichen planus is a common muco-cutaneous disorder, more frequent within the oral cavity, where it appears as either white reticular, or erosive lesions. (1) In the majority of patients with oral lichen planus (OLP) there is no associated cutaneous lichen planus or lichen planus at other mucosal sites. This may be called "isolated" OLP. (2) Although the exact etiology is unknown, it is generally considered to be an immunological hypersensitivity reaction, characterized by an intensive T-cell infiltrate localized in the epithelium-connective tissue interface. (3) However, there are various factors that may predispose to its etiopathogenesis. Most patients with lichen planus are middle-aged or older adults. Women predominate in most series of cases, usually by a 3:2 ratio over men. (1) The oral lesions are reticular, papular, bullous, plaque --like, atrophic, erosive and ulcerated. A rarely encountered form of lichen planus is the bullous variant. (4) Lesions are usually seen on the buccal mucosa and less common on the tongue, inner aspect of the lips and gingiva. (5) Gingival manifestations of lichen planus are relatively rare. Lichen planus is often diagnosed based on clinical information only, but erosive and bullous variants of lichen planus always require laboratory evaluation. (6) Histological examination, immunohistology, particularly immunofluorescence, is increasingly being used to more accurately diagnose such diseases. Direct immunofluorescence analysis is not only proving very useful for differential diagnosis, but also adds insight into possible pathogenic mechanisms of desquamative gingivitis and it is essential for diagnosis of lichen planus. (6) Early recognition of lichen planus or the vesiculobullous disorders may prevent delayed diagnosis and inappropriate treatment of potentially serious dermatological diseases. (7) Treatment includes correction of predisposing factors and the use of topical steroids. However when the mainstay of treatment fails, systemic steroids, various immunomodulatory agents, Retinoids and Thalidomide may also be used. (1)
A 60 year-old patient reported to department of periodontics with the complaint of burning sensations in the gums for the past 09 months. History reveals that patient was asymptomatic 09 months back when she developed burning sensations in the maxillary gingival region. The burning sensations were particularly increased on intake of hot and spicy foods. History also reveals that the patient was under depression for some personal family reasons. There was no associated history of vesicle formation in any area of oral cavity. Extra oral examination reveals multiple papular lesions on the skin of lower back region with no relevant drug history or family history. (FIG. 1) On Intra-oral examination, there was area of desquamation involving marginal and attached gingiva in relation to maxillary and mandibular anterior teeth. The desquamated area was surrounded by minute radiating whitish striations. The desquamated area showed bleeding on probing. Gingiva was soft and edematous in consistency. No periodontal pocket and mobility was observed on periodontal examination. (FIG 2). Non scrappable, whitish interlacing, radiating striae (wickham's striae) were seen on lower labial mucosa, right & left buccal mucosa. (FIG 3, 4 & 5). Melanin pigmentation was also seen on left buccal mucosal lesions. Whitish plaque like lesion was observed on the dorsum of tongue. (FIG.6). Considering the history of 9 month duration, areas of desquamation with radiating white striations without formation of vesicle, along with charactestic skin lesions, provisional diagnosis of Lichen Planus can be given. After the patient was informed about the disease and getting her approval, incisional biopsy was performed. Histopathological features were consistent with the diagnosis of Lichen Planus. (FIG 7). The patient was subjected for thorough oral prophylaxis and oral hygiene instructions. Thereafter, the patient was prescribed topical application of high potency steroids (Clobetasole propionate) 3 times daily for one month. Tablet Cetzine (antihistaminic) once daily for 15 days and once daily tablet of chelating agent (Supracal) was also given to the patient. The patient was reviewed every 2 weeks for the first one month. The patient was referred to dermatologist for the cutaneous lesions. The lesions had subsided with topical steroids within 4 weeks of starting the treatment.(FIG 8, 9, 10, 11) The patient was asked to stop the topical application and reinforcement of oral hygiene instructions was given. Since the lesions can recur, the patient was under observation for 6 months and the lesions showed no signs of recurrence.
Oral lichen planus is a relatively common mucocutaneous inflammatory disease affecting 1% to 2% of the population. (1) The term "Lichen Planus" was coined by the British physician Erasmus Wilson in 1869. (8) Since Lichens are primitive organisms of symbiotic algae and fungus, and planus in latin refers to "Flat". Most patients who experience this disorder are middle-aged or elderly, and 60 % are female. (3)
The exact etiology is not known. (2) However, OLP is a T-cell mediated auto-immune disorder in which auto-cytotoxic CD8 T cells triggers the apoptosis of oral epithelial cells, leading to chronic inflammation. (8)
1) HEPATITIS C VIRUS Infection: HCV
infection is more common in Erosive Lichen Planus. HCV viral sequences have been found in the serum of patients with OLP; and HCV was shown to occasionally replicate in Oral Lichen Planus tissue, possibly contributing to the pathogenesis of mucosal damage. (9,10)
2) PSYCHOLOGICAL FACTORS:OLP patients exhibit higher levels of anxiety, depression, stress and psychological disorders. The levels of anxiety and salivary cortisol of OLP patients are high, thus establishing the relationship of OLP and stress. (9)
3) ORAL LICHENOID REACTIONS:
a) Dental restorative materials: Amalgams, composite resins, cobalt, gold and even flavouring agents may lead to oral lichenoid reactions. (9)
b) Drugs: NSAIDS, ACE inhibitors( captopril), beta blockers, Penicillamine may be implicated as a cause. However, lichenoid reactions are usually unilateral and resolves on discontinuation of the offending factors. (11)
4) MECHANICAL TRAUMA: Dental procedures, friction from sharp cusps, rough dental restorations, poorly fitting prosthesis and deleterious oral habits are exacerbating factors.KOEBNERS PHENOMENON, where the lesions develop in response to trauma, explains why erosive lesions are common in areas subjected to trauma.(buccal mucosa and lateral border of tongue. (9,12)
5 PLAQUE AND CALCULUS: May result in worsening gingival lesions LP and are associated with a higher incidence of erosive lesions.
6 DIABETES AND HYPERTENSION: There is no literature supporting the association of LP with Diabetes and Hypertension. However GRINSPAN'S SYNDROME is the association of LP, Diabetes and Vascular Hypertension. (13)
A) EXTRA-ORAL MANIFESTATIONS:
1) CUTANEOUS LESIONS: consists of purple, pruritic, polygonal papules, often overlined by radiating lines (WICKHAM'S STRIAE). (13) Skin lesions develop several months after the appearance of oral lesions and are usually self limiting. Genital mucosa is the most common extraoral site of involvement.
--is the association of LP of vulva, vagina and gingiva in female patients. Patients usually complains of burning, pain, vaginal discharge dyspareunia. (9)
PENO-GINGIVAL SYNDROME--is the male counterpart of vulvovaginal-gingival syndrome of LP. (9)
2) SCALP AND HAIR FOLLICLES: Lichen planopilaris/Graham's little syndrome represents LP involvement of scalp and hair follicles, resulting in scarring alopecia. (9)
3) NAILS: Thinning and ridging of the nail plate and splitting of the distal free edge of the nail.Healing with scar produces PTYERGIUM. (9)
4) ESOPHAGEAL LESIONS: Dysphagia is the commonest feature. Chronic pain and strictures may also be seen.
B) ORAL MANIFESTATIONS:
The clinical evaluation of the oral lesions is based on the six clinical forms described by Andreason: reticular, papular, plaque, atrophic, erosive, and bullous. (14) Mucosal lesions, which are multiple, generally have a symmetric distribution, particularly on the mucosa of the cheeks, adjacent to molars, and on the tongue mucosa, less frequent on the labial mucosa (lichenous cheilitis) and on the gums (desquamative gingivitis). (14) The most common form is reticular type with characterstic slender white radiating interlacing striations. (15) The lesions frequently occurs bilaterally and are mostly asymptomatic. (15) Erosive LP most often appears as a mixture of intensely erythematous mucosa with large areas of irregularly shaped ulceration with a whitish-yellowish pseudomembrane. (4) Erosive and atrophic LP results in burning sensations. Erythematous lesions that affect the gingival cause desquamative gingivitis, the most common type of gingival LP. The plaque like forms of LP may resemble leukoplakia, particularly proliferative verrucous leukoplakia and appears as slightly raised or flat area on oral mucous membrane. (16) The most common site of plaque like LP is tongue. Bullous LP is extremely rare form in oral cavity. The bullae rupture almost immediately, leaving an ulceration on a bed of inflamed mucosa. Bullous LP most commonly affects the posterior buccal mucosa. (16)
The most important complication of OLP is the development of Oral squamous cell carcinoma. (17) The first case of carcinoma arising in LP of oral mucosa was described by HALLAPEAU in 1910.18 The risk of malignant transformation varies from 0.4% to over 5% a period of 5-20 years. Accumulation of inducible nitric oxide synthetase with 8nitroguanine and 8-oxo-7, 8-dihydro-2' deoxyganosine in oral epithelium in OLP may cause oxidative and nitrative damage to DNA and could be the basis of malignancy. The risk of malignant transformation is independent of the clinical type of OLP or the treatment used. (19)
1) CLINICAL DIAGNOSIS: is sufficient to establish a diagnosis of OLP, if characterstic oral and skin lesions are present.
2) HISTOPATHOLOGY: (9)
--superficial band like infiltrate of T lymphocytes
--Basal cell liquefaction degeneration
--Normal epithelial maturation pattern
--SAW TOOTH rete pegs
--Separation of epithelium from lamina propria
--Max joseph spaces
3) IMMUNOFLUORESCENCE OF PERILESIONAL MUCOSA: 9
--Fibrin and shaggy fibrinogen in a linear pattern at basement membrane zone
--Cytoids in the absence of deposition of fibrinogen
Generally, no medication is necessary for the benign form of this disease (reticular lichen planus). In the case of severe pain and a burning sensation, high-potency topical corticosteroids remain the most re-liably effective treatment modality. Oral hygiene and corrective dentistry play a major role in the management of OLP and consultation with a dentist or oral medicine specialist is helpful. (9)
Drug treatment with topical steroids is preferred due to fewer side effects. Systemic steroids may be used if the lesions are extensive, or there are recalcitrant disease.
Most effective topical steroids are the medium potent steroids (triamcinolone), high potent steroids (fluocinolone acetonide) and superpotent halogenated steroids (clobetasol propionate). Elixir forms are also used such as dexamethasone, triamcinolone and clobetasol. These are used for diffuse oral involvement, elderly patients or for patients having difficulty in applying the medications. The greatest difficulty in using topical steroids is is the lack of mucosal adherence for a sufficient period of time. Therefore, topical steroids may be used with adhesive pastes (orabase). (9) A regular follow up should be done for prolonged use of topical steroids for the following adverse effects:
a) secondary candidal infection
b) Tachyphylaxis (diminished biological effectiveness)
c) Adrenal suppression
d) atrophy of the oral mucosa
Used for intractable erosive OLP lesions. Triamcinolone acetonide (10-20 mg/ml) is used and repeated every 2-4 weeks. Frequent steroid injections are painful and may result in an unwanted systemic dose. (9)
Should be reserved for recalcitrant cases of Erosive or Erythematous OLP or for widespread OLP with skin, genital, scalp or esophageal involvement. Daily doses of 40-80 mg is usually sufficient to achieve a response. (9)
OTHER TOPICAL AGENTS:
a) TOPICAL CYCLOSPORINE (100 mg/ml as a mouth rinse)--may be beneficial in recalcitrant OLP cases. Systemic absorption is generally low, but it is expensive and less effective than topical steroids in inducing clinical improvement in OLP. (9)
b) TOPICAL TACROLIMUS: is a steroid free topical immunosuppressive agent. It was used primarily for atopic dermatitis. The exact mode of action is not known they inhibit T cell activation and proliferation. Burning is the most common side effect with tacrolimus. Other side effects include-carcinogenicity, mutagenesis and infertility. (20)
c) TOPICAL RETINOIDS: Tretinoin and iso treinointrophic are used for erosive-atrophic forms. Side effects include teratogenicity and liver dysfunction.
--PUVA THERAPY--Photochemotherapy with 8 methoxypsoralen and long-wave ultraviolet light (PUVA) has been used successfully in the treatment of skin lesions and cu-taneous lichen planus. (21,22) It was first used in the treatment of recalcitrant OLP. (23) Eighty-seven percent of patients treated with ultraviolet-A, without a sys-temic or topical photosensitizer, improved signifi-cantly. (24) Some studies have indicated that PUVA therapy might also have therapeutic effects. (23) To avoid PUVA side effects, photosensitization with topical 0.01% trioxsalen can be used for the treat-ment, 25 although oral mucosa seems more resistant to phototoxic damage in comparison to skin. (26) PUVA with 8-methoxypsoralen has various side effects, such as nausea, dizziness, eye symptoms, paraesthe-sia, and headache. (27) Photochemotherapy may be use-ful for severe forms of erosive OLP that do not re-spond to conventional treatment. (28) Moreover, one matter of concern is that PUVA therapy has been shown to have oncogenic potential. (29)--CRYOSURGERY--has also been used, particularly in erosive drug resistant OLP, but the lesions may develop in healing wounds and result in scars.
--LASER THERAPY--CO2 lasers are used to treat multicentric lesions or lesions in difficult areas.
ADDITIONAL DRUG THERAPY:
Griesofulvin, Thalidomide, Levamisole and Dapsone may also be used.
Resection my be done for isolated plaques or non-healing erosions. Free soft tissue grafts have been used for localized areas of erosive OLP. However, periodontal surgery have been reported to provoke OLP.
CONCLUSION: OLP are a relatively common oral mucosal disease process encountered in clinical practice. Given the apparent risk of oral SCC, regular follow up of these patients is mandatory.
[FIGURE 1 OMITTED]
[FIGURE 2 OMITTED]
[FIGURE 3 OMITTED]
[FIGURE 4&5 OMITTED]
[FIGURE 6 OMITTED]
[FIGURE 7 OMITTED]
[FIGURE 8 OMITTED]
[FIGURE 9 OMITTED]
[FIGURE 10&11 OMITTED]
(1.) Vincent SD, Fotos LPG, Baker KA, Williams TP. Oral lichen planus: the clinical, historical, and therapeutic features of 100 cases. Oral Surg Oral Med Oral Pathol 1990; 70: 165-171.
(2.) Al Hashimi I, Shifter M, Lockhart PB, Wrap D, Brennan M, Migliorate et al :OLP and Oral lichenoid lesions OOO S25;e; 2007; 103: 1-12.
(3.) Sapp JP, Eversole LR, Wysocki GP: Contemporary Oral and Maxillofacial Pathology, Second Edition, Mosby, St.Louis, 2004.
(4.) Kuffer R; Lombardi T; Erosion and ulcerations occurring in oral lichen planus; Dermatology 2003; 207(3): 340.
(5.) Dissemond J. Oral lichen planus: an overview. J Dermatolog Treat 2004; 15(3): 136-140.
(6.) Raghu AR, Nirmala NR, Sreekumaran N: Direct immunofluorescence in oral lichen planus and oral lichenoid reactions. Quintessence Int. 2002; 33(3): 234-239.
(7.) Mignogna MD, Lo Russo L, Fedele S: Gingival involvement of oral lichen planus in a series of 700 patients. J Clin Periodontol.2005; 32(10): 1029-1033.
(8.) Axell T, Rundquist L. Oral lichen planus-a demographic study. Community Dent Oral Epidemiol. Feb 1987; 15(1): 52.
(9.) D Eisen; M Carrozzo; J-V Bagan Sebastian; K Thongprasan :Oral lichen planus: Clinical features and management: Oral diseases (2005); 11: 338-349.
(10.) Lodi G; Porter SR; Hepatitis c virus and OLP: A short review: oral diseases: 1997; 3: 77-89.
(11.) Dinkova AT; D Gospidava--Interdisciplinary approach in complex treatment of Oral lichen planus-Review and case report-Journal of IMAB.
(12.) Katta R;Am Fam Physicians 2000; 63: 3319-3324.
(13.) A Text book of oral pathology--Shaffers; Hine; Nevy; 5 th edition.
(14.) Andreason JO; Oral lichen planus--A clinical evaluation of 115 cases; OOO; 1968; 25: 31-42.
(15.) Lozada-Nur F, Miranda C: Oral lichen planus: epidemiology. Clinical characteristics, and associated diseases. Semin Cutan Med Surg. 1997; 16(4): 273-277.
(16.) Scott S; De Rossi; Katherine N Ciarrocca: Dental clinics of north America 2005; 49: 77-89
(17.) Meij van der EH, Reibel J, Slootweg PJ, Wal JE van der. Interobserver and intraobserver variability in the histologic assessment of oral lichen planus. J Oral Pathol Med 1999; 28: 274-277
(18.) Hallopeau H. Sur un cas de lichen de Wilson gingival avec neoplasie voisine dans la region maxillaire. Bull Soc Fr Derm Syph 1910; 17: 33
(19.) Chiayarit P; Jontell M; Hiraku Y et al; Nitrative and Oxidative damage of DNA in OLP; Cancer Sciences; 2005; 96; 553-559
(20.) Nazzaro G; Cestari R; ;Topical Tacrolimus ointment in ulcerative lichen planus; An alternative therapeutic approach; European journal Dermatology july 2002; 12: 4; 321
(21.) PUVA treatment in lichen planus: comparison of oral and external methoxsalen regimens. Photodermatol 1987; 4: 265-268
(22.) Jansen CT, Lehtinen R, Happonen RP, Lehtinen A, Soderlund K. Mouth PUVA:new treatment for recalcitrant oral lichen lanus. Photodermatol 1987; 4: 165-166
(23.) Chen HR. A newly developed method for treatment of oral lichen planus with ultraviolet irradiation. Taiwan Yi Xue Hui Za Zhi 1989; 88: 248-252.
(24.) Lehtinen R, Happonen RP, Kuusilehto A, Jansen C. A clinical trial of PUVA treatment in oral lichen planus. Proc Finn Dent Soc 1989; 85: 29-33.
(25.) Kuusilehto A, Lehtinen R, Happonen RP, Heikinheimo K, Lehtimaki K, Jansen CT. An open clinical trial of a new mouth-PUVA variant in the treatment of oral lichenoid lesions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997; 84: 502-505.
(26.) Kuusilehto A, Lehtinen R, Jansen CT. Comparison of the minimal phototoxic dose in topical 4, 59, 8-trimethylpsoralen PUVA treatment of Caucasian skin and of oral mucous membrane. Acta Derm Venereol 1990; 70: 508-509.
(27.) Lundquist G, Forsgren H, Gajecki M, Emtestam L. Photo-chemotherapy of oral lichen planus. A controlled study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995; 79: 554-558.
(28.) Seoane J, Vazquez J, Romero MA, Aguado A, Pomareda M. [Photochemotherapy in the treatment of oral erosive lichen planus. Letter]. Acta Otorrinolaringol Esp 1997; 48: 251-253.
(29.) Lindelof B, Sigurgeirsson B, Tegner E, Larko O, Johannesson A, Berne B, et al. PUVA and cancer: a large--scale epidemiological study. Lancet 1991; 338: 91-93
Sameer Ahmed , Shamimul Hasan , Abhishek Singhvi 
 Assistant professor
Department of Periodontics, Vyas Dental College and Hospital, Jodhpur
 Assistant professor
Department of Oral Medicine and Radiology, Jamia millia Islamia, New Delhi
 Assistant professor
Department of Oral pathology, Vyas Dental college and Hospital, Jodhpur
Email for correspondence:
Received: April 14, 2012; Review Completed: May, 13, 2012; Accepted: June 12, 2012
Published Online: August, 2012 (www.nacd.in)
|Printer friendly Cite/link Email Feedback|
|Title Annotation:||CASE REPORT|
|Author:||Ahmed, Sameer; Hasan, Shamimul; Singhvi, Abhishek|
|Publication:||Indian Journal of Dental Advancements|
|Article Type:||Clinical report|
|Date:||Apr 1, 2012|
|Previous Article:||Bimaxillary protrusion--management with sliding mechanics.|
|Next Article:||Verrucous carcinoma in the oral cavity --report of two cases.|