Lupus Erythematosus: Analysis of Cases

Lupus is a chronic, autoimmune disease that damages any part of the body such as skin, joints or any part inside the body. Autoimmune disease means that our immune response cannot differentiate foreign invaders and the healthy tissue in the body. Thus the body creates autoantibodies that will attack the body’s healthy tissue. Most of the people that being affected are woman as 9 out 10 people who have lupus are woman. It commonly develops in woman between the ages of 15 to 45. It can be difficult to distinguish lupus from other disease as it can mimic many other diseases and often the symptoms will develop slowly as no pattern can be detected. It also can come and go away. Early detection is always the best way to lessen the progression and severity of the disease. Lupus can be caused by few factors such as genes, environment, hormones. There are no genes to be scientifically proven to cause lupus but the incidence of having lupus on family member give the idea of genes does contribute to lupus disease. Lupus can also be triggered by some environmental factors such from the uv rays from the sun, antibiotic drugs like penicillin, sulfa drugs which make a person more sensitive to sun, an infection, injury or even a cold or viral illness. Regarding hormones, sex hormones can play a role in developing lupus particularly estrogen. This hormone is produced by both male and female but the production is high in female which may indicate the severity of lupus. Lupus is categorized into three different types that are:

1. Systemic lupus erythematosus (SLE) by which always referred as lupus. It involved systemic that means it can affect many types of organs or parts of the body. A person with SLE can have mild to severe symptoms.
2. Discoid lupus erythematosus that only affect the skin. A person will have red, raised rash on the face, scalp or elsewhere. Those areas will become thick and scaly.
3. Drug induced lupus which refer to lupus that caused by the use of medications. Usually the symptoms will stop after the medication stopped.

Case report

A 70 year old woman was reported to the Department of Periodontology of Adesh Institute of Dental Sciences & Research with chief complaint of dry mouth, fever and generalized redness of gums with associated burning sensation in the gum for one month which had started spontaneously. She also had complaint of bleeding gums for the past one month while brushing and eating. Her medical history revealed that she was diagnosed with systemic lupus erythemathosus about 10 years ago. Her vital signs were monitored which showing normal. Clinical examination revealed facial cutaneous depigmented malar rash.

Her laboratory examination showed WBC count at 2300 (neutrophils 30%, lymphocytes 45%, monocytes 25%), high ESR and normal urinalysis. Intraoral examination revealed that there are presence of erythematosus marginal, attached gingiva and interdental papilla in maxillary and mandibular anterior region. Nikolsky’s sign showed positive. Generalized bleeding on probing was positive; however no periodontal pockets or furcation involvement was seen.

Clinical diagnosis was established by incisional biopsy, performed under local anesthesia. Histological examination reveals the findings that are consistent with SLE. So the management for this patient in periodontal department is scaling as gently as possible as well as advise to maintain the oral hygiene. She was prescribed with soft bristle toothbrush with Triamcinolone acteonide oral paste and Chlorexhidine mouthwash.

A 68 year old male presented with a complaint of non healing ulcers in the mouth since 6 months associated with pain and burning sensation. Gradual onset associated with erythema and burning sensation followed by blistering that ruptured within 2-3 days led to ulceration of the region. There was no prodromal fever or malaise. Initially lesions were noted over the dorsum of the tongue followed by the involvement of the palate and buccal and labial mucosa associated with difficulty in swallowing. Two months later, he noticed similar lesions over the skin, predominantly on the trunk and scalp regions, which healed within 7-14 days.

The patient is under skin consultation and currently taking topical corticosteroids and anticandidal mouth rinses. Skin lesion showed signs of healing but intra oral lesion persistent. Medical history revealed that the patient is known with diabetic since 14 years and is under medication. Family history is not significant. Patient was poorly nourished and weak with signs of pallor.

Examination of the trunk revealed multiple, well defined, roughly round erosive lesions measuring approximately 1x2 cm in dimension. Head and neck examination showed discrete erosive lesions over the cheek, nose and scalp measuring approximately 0.5 x 1cm in diameter. Scalp lesions causing scarring. The bilateral submandibular lymph nodes are palpable, single in number, tender, firm, mobile and measured 1cm in diameter.

Intraoral examination revealed painful restricted mouth opening with multiple superficial discrete ulcerations along the upper and lower buccal mucosa, labial mucosa, soft palate and vestibule. Diffuse, irregular ulcer covered by a pseudomembraneous slough were noted. On palpation, the ulcers are tender and bleed on slight provocation. Multiple fibrotic bands are felt along the bilateral buccal mucosa and circumorally. The tongue seems to be depapillated, with areas of fissuring and erythema. Hard tissue examination was done revealed poor periodontal health, with generalized mobility and attrition of the teeth.

Histopathological section was obtained from the incisional biopsy of a skin lesion that revealed epidermal hyperkeratosis and focal keratotic plugging. It also can be seen mild atrophy of the stratum malphigii and mild degeneration of the basal layer. It suggests DLE as the dermis showed mild edema and few scattered aggregates of mononuclear chronic inflammatory cells in the form of lymphocytes and plasma cells.

The treatment given to this patient on the first visit was given to her the topical antibiotics and analgesics along with multivitamins, antioxidant supplement and protein, topical corticosteroid was given for the skin lesions. Follow up was done after 5 days.

A 19 year old male patient, work as farmer came with the chief complaint of painful ulcer on his lower lip since 6 months ago. The nature of the pain was insidious in onset, localized throbbing to pricking type which is moderate intensity aggravates on taking food or trauma to that region. The ulcers were initially small in size and gradually progressed to the present size with the incidence of white serous discharge. Lymph node were non palpable. Systemic evaluation was done and no significant evidence contributes. The patient had class 3 facial profile with lower lip protuberance. Two discrete ulcers which are oval in shape measuring 1cm x 1cm with regular borders with the thin marginal zone of erythema. There was a presence of radiating striae from the zone of erythema. Considering the oral manifestation, a provisional diagnosis for the mixed white and red lesions was given. The differential diagnosis given was DLE actinic keratosis and erythema multiforme minor.

The treatment given was corticosteroid therapy systemically and topically for the duration of 21 days. The patient was advised to cover the mouth with a soft cloth while he is out in the fields. It is reaffirmed that the diagnosis is DLE based on the clinical presentation, the nature of the lesion and the reponse towards treatment.

Discussion

Systemic lupus erythematosus is an autoimmune disease that comes with many cutaneous and oral manifestations. As mentioned before, the disease attack whenever the body produces autoantibodies to many of their cells and cell components and tissues. Hormone and gender play a vital role in SLE as it is more common in females and always correlate to the production of estrogen and progesterone levels. SLE often appears in no pattern. Clinically, lupus is a disease with an unpredictable course involving the flares and remissions, where the longer the time it progress the more damage it give to the body.

The common symptoms that can occur to a person with SLE are extreme fatigue, headache, fever, swollen joints, anemia, pain in chest on deep breathing, butterfly shaped rash across cheek and nose, sun or light sensitivity, hair loss, abnormal blood clotting and mouth and nose ulcers. Mucosal involvement as in patient with SLE is common. It typically seen as a well demarcated erythema or erosion with central white papules surrounded by white radiating striae. A study was done looking at oral findings in patients attending the multidisciplinary lupus clinic between January 2015 and April 2016. The results are most of the patients are female (88%) and had diagnosis of DLE (62%), 26% had SLE. Half of the patients had positive oral finfings, 26% had no oral examination documented and 24% had oral examination documented. The commonest site of oral findings are at the palate. Then at the labial mucosa, buccal mucosa, gingiva and alveolar ridge. Oral manifestations that were consistent with lupus were erosion or ulcers (5cases), erythema in 4 cases and a white plaque in 4 cases. In this cohort study it is normal to find oral pathology and it is compulsory to do oral examination to identify oral lupus and provide the treatment.

Fabri et al revealed that the treatment of periodontal disease is beneficial in controlling disease activity among SLE patient with immunosuppressive therapy. A recent study also said that periodontal treatment can be helpful in reducing the symptoms in SLE.

DLE is a type of lupus erythematosus that usually confined to the skin and has minimal systemic involvement. The lesions usually appear as eryhthematous, edematous, scaly papules that spread centrifugally and coalesce into plaques. Lifting of the scales produces a carpet-tack appearance then revealing dilated pilosebaceous orifices occupied by horny plugs. The healing of a lesion will takes place in the center, producing atrophy, scarring, telangiectasia and pigmentary changes. Scarring alopecia is a significant finding. Mucosal findings usually affected about 24% of the DLE patients appearing as chronic plaques or lichen planus-like oral lesions, ulcerations, cheilitis and plaque=like palatal lesions.

DLE patients may manifest any symptom of SLE that why assessment for symptoms of pleuritis, pericarditis, neurologic involvement and renal involvement. The diagnosis of DLE can be obtained from biopsy taking from an established lesion. Topical steroid will always be the first line of treatment for the localized cutaneous and mucosal lesion. There also intralesional cortisone injections, systemic corticosteroid, calcineurin inhibitors, pimecrolimus cream or tacrolimus ointment, aminoquinoline antimalarials, dapsone or imiquimod 5% may be used for antifungal therapy topically. Follow up is very crucial and necessary for every 6 months as DLE is considered as a pre-cabcerous condition and also for early detection of SLE.

References

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