Background

Lupus erythematosus (L.E) is a chronic autoimmune disease in which auto-antibodies bind to components of the cell surface, cytoplasm and nucleus, including nucleic acids and nucleoprotein particles. This antibody binding prompts an inflammatory reaction, which leads to cell and tissue destruction (Louis and Fernandes, 2001).

L.E occurs most commonly in young women and ranges from mild cutaneous lesions and ⁄ or arthritis to renal failure or intense nervous, cardiac and hematological disturbances (Neville et al., 2009).

L.E can manifest as different clinical forms: systemic lupus erythematosus (SLE), chronic cutaneous lupus erythematosus and subacute cutaneous lupus erythematosus (Burge et al., 1989).

A wide spectrum of oral mucosal lesions is found in cutaneous and systemic forms of LE: cheilitis, erythematous patches, ‘honeycomb’ plaques, discoid lesions, lichen planus (LP)-like lesions and discrete ulcers have been described. In established disease, reported prevalence rates range from 9% to 45% of cases (Jonsson et al., 1984; Rowell and Goodfield, 1998) although the true figure may be nearer to 20% (Lever et al., 1983).

1 Case Report

A 33 year old female patient came to the department of Oral Patholgoy and Microbiology, Bharati Vidyapeeth Dental College, with the chief complaint of ulcers and burning sensation in the oral cavity since last 6 months. She also complained of pain in the joints of the lower limbs. Extra oral examination and facial appearance appeared normal (Figure 1).

Intraoral examination revealed reddish ulcers on the buccal mucosa, tongue, soft and hard palate (Figure 2, Figure 3, Figure 4 and Figure 5). Patient had visited a private dentist for the same and was prescribed mouthwash and anti-inflammatory drugs with vitamins supplements but the lesion was not responding to any drug. Our differential diagnosis was Lupus Erythematosus and Lichen Planus. Patient was advised investigations like routine hemogram, Xray of the bone, and biopsy with anti-nuclear antibody (A.N.A) testing immuno-fluoroscence.

A biopsy of the representative lesion was taken under Local anesthesia under antibiotic coverage. Hemotoxylin and Eosin (H.E) stain section showed stratified squamous epithelium with hyperkeratosis and acanthosis (Figure 6), perivascular cuffing, liquefaction degeneration of basal cell layer (Figure 7) and chronic inflammatory cells as well as sub-epithelial vesicles (Figure 8). ANA and immune-fluroscence tests were positive (Figure 9 and Figure 10). Patient was referred to the general physician for further investigation opinion and treatment.

2 Discussion

SLE has a complex etiopathogenesis including genetic factors, drug induced, viral infection, sun exposure and estrogen. Clinical features of SLE are diverse as it affects joints, lungs, nervous system, kidneys and skin. The initial complaint starts with fever, malaise, joint pain, muscle pain and fatigue. While SLE is more common in females than males, the symptoms associated with each sex are different. Females tend to have greater relapses, decreased WBCs, Raynaud’s phenomena, arthritis and psychiatric symptoms while males have kidney diseases, serositis, skin rash and peripheral neuropathy (Long et al., 1998).

Inflammation caused by SLE may affect many parts of the body. Anemia is a common complication along with problems in bleeding and clotting time and vasculitis. Patients with SLE are more susceptible to inflammation of the lung. Many people with lupus complain of headache, dizziness, behavioural change and memory problems. Kidney damage is a common complication and may lead to death. The risk of cardiovascular diseases and heart attack is more common in SLE patients. Many studies suggested increased risk of cancer in SLE patients. Women with lupus have increased risk of miscarriage. In the oral cavity, long term use of medication to control lupus can cause root canal calcification, delayed tooth eruption, dilacerations of root, gingival enlargement with bleeding and viral infections (Burnham et al., 1963).

The main differential diagnoses are Lichen Planus (L.P) and oral leucoplakia, but distinguishing between oral LE, LP and leucoplakia can be difficult, both clinically and histologically, even when established histopathological criteria are used (Bottomley and Goodfield, 1995).

The American College of Rheumatology has published criteria for the classification of patients as having SLE (Figure 11). If a patient has, at any time in his or her medical history, 4 of the 11 criteria documented, the diagnosis of SLE can be made with approximately 95% specificity and 85% sensitivity.

Treatment is aimed at symptom control. There are very few large, controlled studies that describe the management of this chronic disease comprehensively. The efficacy of methotrexate (Ruzicka and Goerz, 1981), dapsone (Edwards and Gayford, 1977) and gold (Gardner-Medwin and Powell, 1994) is described in isolated cases and small series.

Thalidomide should be of benefit on theoretical grounds (Cojocaru et al., 2011). Antimalarials (hydroxychloroquine and mepacrine) and azathioprine in patients with oral manifestations of LE, LP and lichen sclerosus, and have found to be of significant benefit.

Expression of immunofluorescence in almost all cases emphasizes the importance of the direct immunofluorescence, differentiating LE with other diseases as oral lichen planus. However, as some cases were negative to any immunological marker, diagnosis should not be relying on immunofluorescence results alone. Clinical, histopathological and immunopathological correlation is imperative in order to accomplish a correct diagnosis (Berbert and Mantese, 2005).

Authors’ contributions

S.L. collected records, observations, investigations and diagnosis, Y.M. edited reading and checked references and R.S. drafted manuscript. There was no conflict of interest. All authors read and approved the final manuscript.

Acknowledgments

The authors wish to acknowledge the contribution of Dr. Swapnil Karnik (M.D) Onco and Renal Histopathologist, (Megavision Labs, Pune, Maharashtra, India) for the immunofluorescent studies. Authors disclose that no financial funding or support was provided by any agency/institute for the the compilation of the case report or manuscript.

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