Oral and cutaneous lichen planus correlate to primary sclerosing cholangitis: a case report
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1
Azienda Ospedaliero-Universitaria di Modena, Surgical, Medical and Dental Department of Morphological Sciences related to Transplant, Oncology and Regenerative Medicine, Italy
Aim. Lichen planus (LP) is a chronic inflammatory and immune-mediated disease with a mucosal and cutaneous involvement. The pathogenesis is characterized by T CD8+ and CD4+ mediated response against epithelial basal cells, leading to basal layer degeneration and sub-epithelial band like T-lymphocytes infiltration. The etiology is still unknown, but it is believed that many environmental agents can trigger the disease in genetically predisposed subjects. These agents include drugs, microbial and viral infections and contact sensitizers. In literature, cases of LP are reported in association with other autoimmune pathologies such as graft versus-host disease, lupus erythematous, primary biliary cirrhosis and Sjögren’s syndrome. The elevated risk of developing LP in patients with chronic hepatopathy, in particular those infected with HCV, has been documented but it is still controversially discussed. The concurrent presentation of primary sclerosing colangitis (PSC) and LP has been previously described in five cases: one nail lichen and four oral lichen planus (OLP).
Materials and Methods. A 67-year-old woman was referred to our clinic by her general dentist for evaluation of oral lesions associated with burning and pain symptoms. The patient reported the presence of a white-red painful fissured lesion, involving tongue and cheeks since 6 months. Fluctuating period of remission and worsening were described, strongly associated with spicy and acid food. Medical history was positive for primary sclerosing colangitis, diagnosed in 1997 and treated with angioplasty. In addition, the patient underwent excision of a pulmonary lobe for bronchial carcinoma and thyroidectomy for Hürthle cells adenoma. Drug history accounts for chronic intake of levothyroxine, potassium canreonate, ranitidine and torasemide. Widespread erythematous areas were highlighted during clinical examination, involving keratinized gingiva, lip vermilion, tongue dorsal mucosa and the buccal mucosa. Deep fissurations were present in the tongue dorsal mucosa, while the buccal mucosa showed the presence of reticular white lesion (Wickham's stripes). Moreover, a scaling erythematous dermatitis was appreciable in patient hands and legs skin with tendency to confluence and central atrophy. Scalp skin and hair follicles were also involved. The dermatitis appeared to be distributed predominantly in the photo-exposed areas. The patient referred the onset of these cutaneous manifestation since December 2008, firstly to hands and later to legs and back. Patient thus referred about a skin biopsy, which was not diagnostic for cutaneous lichen. An incisional biopsy of the buccal mucosa was performed.
Results. Histologic examination showed sections of malpighian mucosa with hyperkeratosis, acanthosis and vacuolization of the basal cell layer associated with the presence of necrotic keratinocytes (civatte bodies) and chronic infiammatory infiltration. The diagnosis of oral lichen planus was determined by the clinical and histological result. To date, the patient has refused to perform a new skin biopsy for diagnosis completion.
Discussion. The reported OLP case describes the common epidemiologic appearance. Average age of OLP onset is the 6th decade, lesion affects women and men with a ratio of 2:1. Clinically, characteristic features are present such as Wickham's striae, well- defined looping and intersecting white lines or striae on a background of minimal substantial erythema, and the typical course with period of remission and exacerbation. The buccal mucosa, the tongue, and the gingiva are the most often affected sites. Approximately two-thirds of patients experience discomfort even if the symptoms are very variable; in addition to a spontaneous manifestation, patients occasionally experience discomfort or pain only upon contact with spicy or acidic foods. Moreover, sense of mucosal roughness, reduced mucosal flexibility, and limited mouth opening are often reported.
Patient medical history is worthy of attention for PSC, a rare cholestatic liver disease characterized by concentric and obliterative fibrosis which evolves in stricturing and dilatation of the biliary tree. In the majority of cases, it will progress to cirrhosis. Men are more affected than women and the prevalence, although is rare, is increasing worldwide. Clinical manifestations are very different from the asymptomatic patient with an accidental diagnosis to jaundice and liver decompensation. Complications are common such as liver failure, hepatobiliary malignancy, bowel cancer and death. The pathogenesis is not well defined, but several mechanism have been proposed like immune signalling dysregulation, increased liver toxins level due to bowel well permeability and damage from toxic bile acids. Despite of pathogenic hypothesis, a genetic and immunological basis has been implicated in presence of non-activated memory T lymphocytes and variable association with the human leucocyte antigen (HLA) complex.The immune system dysregulation seems to have a key role in both PSC and LP but the connection of the two pathologies is not certain.Otherwise, the association between liver diseases and LP is well documented. Specifically for OLP, an increasing number of reports suggests a chronic liver disease and oral lesion relationship; conversely, the link between cutaneous lichen planus and liver diseases still remain controversial and not all the literature is unanimous. In spite of this, lichen planus is known to be the most frequent skin manifestation of primary biliary cirrhosis where the exposure to penicillamine seems to be a triggering factor for the cutaneous lesion. PSC is also a frequent finding in association with inflammatory bowel disease, such as ulcerative colitis and Crohn’s disease, which are also described in relation to OLP. In conclusion, few data concerning the correlation of OLP and PSC are present in literature. This clinical case describes a clinical scenario which is similar to existing reports. More studies should be performed to evaluate the role of the immune system in this pathology and the potential correlation between lichen planus and liver diseases.
References
1. Tong DC, Ferguson MM “Concurrent oral lichen planus and primary sclerosing cholangitis.” British Journal of Dermatology (2002), 147: 356–358.
2. Williamson KD, Chapman RW “Primary sclerosing cholangitis:a clinical update.” British Medical Bulletin, (2015),1–12
3. Terziroli Beretta-Piccoli B, Invernizzi P, Gershwin ME, Mainetti C “Skin Manifestations Associated with Autoimmune Liver Diseases: a Systematic Review.”Clinical Reviews in Allergy and Immunology (2017), 53(3): 394-412
Keywords:
Oral lichen planus (OLP),
Cutaneous lichen planus,
Primary sclerosing cholangitis (PSC),
Immune System,
Liver disease
Conference:
5th National and 1st International Symposium of Italian Society of Oral Pathology and Medicine., Ancona, Italy, 19 Oct - 20 Oct, 2018.
Presentation Type:
Poster Presentation
Topic:
Oral Diseases
Citation:
Tognacci
S,
Setti
G,
Lizio
G,
Consolo
U and
Bellini
P
(2019). Oral and cutaneous lichen planus correlate to primary sclerosing cholangitis: a case report.
Front. Physiol.
Conference Abstract:
5th National and 1st International Symposium of Italian Society of Oral Pathology and Medicine..
doi: 10.3389/conf.fphys.2019.27.00073
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Received:
05 Nov 2018;
Published Online:
09 Dec 2019.
*
Correspondence:
Dr. Sara Tognacci, Azienda Ospedaliero-Universitaria di Modena, Surgical, Medical and Dental Department of Morphological Sciences related to Transplant, Oncology and Regenerative Medicine, Modena, Emilia-Romagna, Italy, sarah.tognacci@gmail.com