CLINICAL CASES
Zuzanna Ślebioda
B–D, Barbara Dorocka-Bobkowska
A, E–F, Elżbieta Szponar
A–B, ECoexistence of Oral Lichen Planus and Vitiligo
– Case Report
Współwystępowanie liszaja płaskiego jamy ustnej i bielactwa nabytego
– opis przypadku
Department of Oral Mucosa Diseases, K. Marcinkowski University of Medical Sciences, Poznań, Poland
A – research concept and design; B – collection and/or assembly of data; C – data analysis and interpretation; D – writing the article; E – critical revision of the article; F – final approval of article
Abstract
The simultaneous occurrence of a number of autoimmune diseases in one patient has been the subject of several reports. This phenomenon may result from similar etiologic pathways involved in the development of the autoim-mune conditions.
In this paper we present a rare coincidence of two autoimmune diseases lichen planus and vitiligo – in a 76-year old patient. To date the etiopathogenesis of lichen planus is not clearly understood and involves both antigen-specific and non-specific mechanisms. Also the pathogenesis of vitiligo is multifactorial and linked to overlapping genetic and non-genetic factors, however the exact pathomechanism of the condition remains elusive. In the described case vitiligo was recognized by a dermatologist based on its characteristic clinical presentation over 30 years ago and remained untreated, while oral lichen planus was diagnosed through a routine clinical examination of the oral cavity, where white non-removable patches and papules producing lacy pattern and located bilaterally on buccal regions, retromolar area and lateral tongue surfaces were revealed. This diagnosis was confirmed in histopathologi-cal assessment of the buchistopathologi-cal mucosa specimen (Dent. Med. Probl. 2014, 51, 4, 541–545).
Key words: lichen planus, vitiligo, oral mucosa, oral lesions.
Streszczenie
W dostępnej literaturze naukowej pojawiały się już prace na temat współwystępowania kilku chorób autoimmu-nologicznych u jednej osoby. Fenomen ten może wynikać ze zbliżonych mechanizmów etiologicznych leżących u podłoża współwystępujących zaburzeń autoimmunologicznych.
W pracy opisano rzadki przypadek dwóch chorób autoimmunologicznych: liszaja płaskiego i bielactwa nabytego u 76-letniego mężczyzny. Jak dotąd etiopatogeneza liszaja płaskiego pozostaje nie w pełni wyjaśniona. Wiadomo jednak, że obejmuje zarówno mechanizmy antygenowo swoiste, jak i nieswoiste. Także patogeneza bielactwa naby-tego wydaje się mieć charakter wieloczynnikowy i związany z nakładającym się wpływem czynników genetycz-nych i środowiskowych, precyzyjna patogeneza choroby nie została jak dotąd jednak jasno określona. W opisanym przypadku bielactwo nabyte rozpoznano w poradni dermatologicznej ponad 30 lat temu na podstawie charakte-rystycznego obrazu klinicznego i od tego czasu nie wdrożono leczenia. Liszaj płaski jamy ustnej stwierdzono na podstawie rutynowego badania stomatologicznego, w którym zaobserwowano obecność białych, nieusuwalnych plam i grudek o siateczkowatym układzie, umiejscowionych obustronnie w okolicy policzkowej, zatrzonowcowej i na bocznych powierzchniach języka. Rozpoznanie to potwierdzono w badaniu histopatologicznym wycinka błony śluzowej jamy ustnej (Dent. Med. Probl. 2014, 51, 4, 541–545).
Słowa kluczowe: liszaj płaski, bielactwo nabyte, błona śluzowa jamy ustnej, zmiany w jamie ustnej. Dent. Med. Probl. 2014, 51, 4, 541–545
ISSN 1644-387X © Copyright by Wroclaw Medical University and Polish Dental Society
The coexistence of a number of autoimmune diseases in a single patient has been previously de-scribed by a number of authors and still remains
a subject of ongoing research [1–3]. The concur-rent conditions have included: autoimmune hepati-tis and ulcerative colihepati-tis, celiac disease and diabetes
mellitus, lichen planus and graft-versus-host disease (GVHD) or primary biliary cirrhosis [2, 4]. In this paper we present a case of a 76-year old male with two concurrent autoimmune diseases: lichen pla-nus and vitiligo. Lichen plapla-nus is a relatively com-mon mucocutaneous disease, affecting between 0.1 and 3% of the general population, with a female predilection [2]. Vitiligo is a pigmentary skin dis-order with 1% worldwide incidence [5]. The back-ground of both lichen planus (LP) and vitiligo re-mains largely unknown, but it is most likely related to some genetically conditioned autoimmune pro-cesses, modified by environmental factors [2, 6].
Case Report
Patient J.N., aged 76, was admitted to the De-partment of Oral Mucosa Diseases in November 2009 due to painless, white lesions on the oral mu-cosa detected during a routine dental visit. He was a non-smoker, he did not report any unusual di-etary habits and was not using any removable den-tal appliance. The family history of similar oral lesions was negative. The medical history of the patient included hypertension and rheumatoid ar-thritis. In 2010 the left lobe of his thyroid gland was removed.
No pathologic findings were observed during the extra-oral examination on the facial skin, how-ever the large, irregularly shaped, non-tender ar-eas of depigmented skin on his hands and feet are shown in Fig. 1. According to the patient, the lesions had persisted for over 30 years and had been previ-ously diagnosed as vitiligo. The size of the patches had remained unchanged for many years. No other body regions were similarly affected and therefore no treatment for vitiligo had been prescribed.
The intra-oral examination revealed white, non-removable patches and papules producing characteristic striae located bilaterally on the buc-cal regions, retromolar area and lateral tongue sur-faces. A small erosion in the stage of healing was also detected on the oral mucosa in the left buccal area. During the visit, the patient reported a mod-erate burning sensation in the mouth.
Dental and radiographic examination showed that several teeth were missing. A panoramic X-ray illustrating the patient’s dental status is presented in Fig. 2.
The teeth remaining in the maxilla included: the third molar and second premolar on the right, first incisor and two last molars on the left. The left and right first and second molars were missing in the mandible. All the upper molars, lower pre-molars and last right molar were filled with
amal-Fig. 1. Depigmented, irregularly shaped patch on
patient’s dorsal hand surface, typical of vitiligo
Ryc. 1. Plamy bielacze o nieregularnym kształcie na
grzbietowej powierzchni dłoni pacjenta charaktery-styczne dla bielactwa nabytego
Fig. 2. Panoramic X-ray
illustra-ting patient’s dental status
Ryc. 2. Pantomograficzne zdjęcie
rentgenowskie ilustrujące stan uzę-bienia pacjenta
gam. The panoramic X-ray revealed the presence of impacted tooth 14.
The differential diagnosis included oral li-chen planus and oral lili-chenoid reaction. Oral le-sions typical of LP, including striae and the ero-sion, were located on the mucosa adjacent to the restorations. The patient received topical anti-in-flammatory treatment with non-steroidal agents, composed of allantoin, herbs (chamomile, mint extract) and salicylates, together with a recom-mendation to improve oral hygiene and to start conservative and prosthetic treatment of the oral cavity. He was advised to remove all the amalgam restorations.
The follow-up visit took place two years lat-er, in April 2011. During the examination, in the left buccal region a small erosion was observed again, which, according to the patient, had not ful-ly healed since the previous visit. The patient was therefore referred for a biopsy of the affected area. An oral mucosa probe from the left buccal region was collected under local anesthesia. Microscopic examination revealed the features of epithelial ac-anthosis and keratosis together with chronic in-flammatory infiltration and damage to the basal cell layer (Fig. 3). The presentation was consistent with the clinical diagnosis of lichen planus.
The patient received topical steroid ointment with dexamethasone and a covering paste com-posed of protein-free calf’s blood extract to apply to the erosion and incised region twice a day for
two weeks. During the subsequent follow-up visit one month later, the lesion had healed and the pa-tient did not report any complaints. The next ex-acerbation of lichen planus was observed in Jan-uary 2013, when bilaterally located erosions sur-rounded by the characteristic white striae were observed on buccal mucosa, which was diagnosed as the erosive type of LP. The patient complained of burning and tenderness of the oral mucosa, which had persisted for a few weeks and was espe-cially evident while eating spicy and acidic foods. Repeated topical treatment with the steroid oint-ment composed of dexamethasone and covering paste with protein-free calf’s blood extract applied twice a day for two weeks resulted in a rapid im-provement.
During the last follow-up visit in November 2013, only an asymptomatic, reticular form of LP was detected, and therefore pharmacological treat-ment was no longer required. The patient howev-er was again urged to improve his oral hygiene and instructed to change all the amalgam fillings to composites. In addition, the extraction of mobile tooth 38 and further prosthetic treatment was also recommended. Due to the coexistence of LP and hypertension, and considering the risk of Grinspan syndrome developing, careful monitoring of blood glucose levels was also recommended in this case.
Reticular lesions on buccal mucosa, as pre-sented in Figs. 4 and 5, were visualized with Schil-ler’s iodine test.
Fig. 3. Buccal mucosa bioptate
present-ing the features of epithelial acanthosis and keratosis together with the chronic inflammatory infiltration and damage of the basal cell layer (H + E stain, 1 × 100)
Ryc. 3. Wycinek błony śluzowej
policz-ka z widocznymi cechami apolicz-kantozy i ke-ratozy wraz z przewlekłym naciekiem zapalnym i uszkodzeniem komórek warstwy podstawnej (barwienie H + E, 1 × 100)
Discussion
To date, the etiopathogenesis of lichen planus is not clearly understood and it involves both anti-gen-specific and non-specific mechanisms that in-clude: CD8+ cytotoxic T cell activity against kera-tinocytes, mast cell degranulation and activation of matrix metalloproteinase [2]. Most probably the disease develops due to a disturbed immune reac-tion with an excessive cell-mediated response. This leads to damage to the basal cell layer in the epi-thelium. The presence of CD4 and CD8 lympho-cytes in mucosal lamina propria in the oral lesions of lichen planus indicate a lymphocytic inflamma-tory reaction to an antigen localized in basal layer keratinocytes [2, 3, 7]. Environmental factors, with a special emphasis on stress, play a crucial role in the induction of the initial episode of the disease
and further exacerbation. Some medications and dental materials, especially amalgams, may also interfere with the condition [8]. The exacerbation of lichen planus may be induced by the presence of arsenic, bismuth and gold compounds, non-steroid anti-inflammatory drugs (NSAIDs) some anti-hy-pertensives and quinidine [9–12]. Some authors have reported frequent coexistence of the triad of diseases: lichen planus, hypertension and diabetes mellitus, described as Grinspan syndrome. The mechanism of this phenomenon requires some further investigation. It has been suggested, how-ever, that antihypertensive and anti-diabetic drugs may induce LP in some patients [13]. As described above, the patient in this paper had suffered from hypertension for many years and was on antihy-pertensive drugs. However, his blood glucose lev-els were found to be normal, hence he did not pres-ent the full spectrum of Grinspan syndrome. The white striae and erosion were located mainly in the patient’s posterior buccal regions, adjacent to large amalgam restorations. Contact hypersensitivity to metal ions could have been a trigger factor for LP in this case. Further observation of the patient’s response after the removal of the amalgams is re-quired to determine whether he suffers from a clas-sic type of LP or rather an oral lichenoid reaction (OLR) – a condition very similar to LP in appear-ance and in histopathological presentation, but in-duced mainly by direct contact with some dental materials, mostly those containing mercury, and by the effects of some drugs. A correlation between OLP and graft-versus-host disease (GVHD) has al-so been reported [8, 9, 11].
The pathogenesis of vitiligo is considered mul-tifactorial and linked to overlapping genetic and non-genetic factors, however the exact pathomech-anism of the condition remains elusive. The pres-ence of circulating autoantibodies and autoreac-tive T cells in vitiligo patients confirms the auto-immune background of the condition [6, 7].
The coincidence of several autoimmune dis-eases in one person was previously mentioned in the literature reports. Autoimmune disorders that may appear together with lichen planus include e.g. graft-versus-host disease (GVHD), primary biliary cirrhosis and type B and C chronic hepatitis [2]. Other authors have reported similar observations that lichen planus may also accompany ulcerative colitis [3, 4, 14]. The coexistence of lichen planus and vitiligo, as in the case described in this paper, was observed by Baghestani et al. [15], Gül et al. [16] and Göktay et al. [17]. They described the colocal-ization of papular skin eruptions, typical of cutane-ous LP, and depigmented patches characteristic of vitiligo. The possible immunological pathway com-mon for LP and vitiligo includes the autoimmune
Fig. 4. Patches and papules producing lacy pattern on
patient’s buccal mucosa
Ryc. 4. Plamy i grudki o siateczkowatym układzie na
błonie śluzowej policzka
Fig. 5. Patches and papules producing lacy pattern
on patient’s buccal mucosa after performing Schiller’s iodine test
Ryc. 5. Plamy i grudki o siateczkowatym układzie na
błonie śluzowej policzka po wykonaniu próby jodowej Schillera
destruction of basal keratinocytes which cross-re-act with the antigens on melanocytes [18]. The co-existence of a number of diseases in one person, in-cluding lichen planus, vitiligo, alopecia, onychodys-trophy and scleroderma, was also found by Cecchi et al. [19] and Brenner et al. [20]. Most of the authors suggested that a combination of genetic susceptibil-ity and environmental factors, which underlie most of the autoimmune conditions, explained the high-er possibility of multiple diseases in one patient [1].
However, the results of a large, cross-sectional study performed recently by López-Jornet et al. [2] do not confirm the higher incidence of autoimmune dis-eases in patients with LP. The authors detected con-comitant autoimmune disease in 7% out of 130 pa-tients with LP and in 4% of 130 healthy controls. Further studies are required to determine whether the suggested association is indeed linked to auto-immune pathogenesis or is coincidental, and to un-derstand the coexistence pathways.
References
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Address for correspondence:
Zuzanna Ślebioda
Department of Oral Mucosa Diseases
K. Marcinkowski University of Medical Sciences Bukowska 70
60-812 Poznań Poland
Tel.: +48 61 854 70 39 E-mail: zuzia_slebioda@o2.pl
Conflict of Interest: None declared Received: 1.07.2014
Revised: 2.07.2014 Accepted: 14.07.2014
Praca wpłynęła do Redakcji: 1.07.2014 r. Po recenzji: 2.07.2014 r.
