Dr F Bhamjee
Oral medicine case report:
Discoid lupus erythematosus
A 59 year old male presented at the oral medicine clinic with a referral note from the department of dermatology. The note requested for the evaluation of intra oral lesions discovered as part of their extensive dermatological examination. They describe the presence white lesions on the buccal mucosa and alveolar ridge particularly on the left side. The patient is generally healthy, a non-smoker , and reports no other medical issues of clinical significance. However his main complaint is the presence of skin lesions of various descriptions affecting multiple sites of his body
Extra-oral examinations:
Reveals multiple lesions affecting the scalp, face , neck , forearms and the patient indicates their presence on the torso and legs. The lesions vary slightly in their descriptions:
Scalp – large white, keratotic plaque like area of skin devoid of hair and all other descriptive factors of healthy skin. The area is rough, dry and leathery to touch, painless and has well defined margins. The marginal area displays an interface between normal skin and hair and an area epithelial regression. Patient indicates it began as a small spot on his scalp that has grown over the past year.
Face – irregular areas skin de-epithelialisation with localised areas of hyperkeratosis, spreading from right to left along the submandibular neck and chin. The lower end of the right earlobe presents with a small stalked pappilomatous growth.
The right forearm presents with a focal area of de-epithelialisation with radiating striae of thinning stratum corneum to smooth hypopigmented areas
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Figure 1: Extraoral lesions
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Figure 2 Intra-oral views
The patient does not understand why the lesions have started but indicated that the first lesion began just over two years ago. A biopsy was taken of the lesions by dermatology but the results were as yet unknown. Intra oral examination revealed the presence of an eplus growth from the palatine ruggae of the hard palate as well as a wide leukoplakia affecting the buccul mucosa and posterior alveolar ridge on the left side. Oral lesions were painless and went unnoticed by the patient.White area appeared similar to lichen planus or leichnoid reaction. Differential diagnosis was difficult to narrow down.Figure 2 Intra-oral views
Incisional biopsy was taken of the affected buccul mucosa and ridge and excisional biopsy taken of the palatal epulis.Differential diagnosis was initially complicated by the presence of the extra oral lesions. In isolation these lesions could be classified as :
- Fibrous epuli of the palate
- Luekoplakia of buccul mucose
- Lichen planus
- Leichnoid reaction
The presence of the skin lesions leads us to consider the likely possibility that there is a systemic condition responsible for both extra-oral and oral mucosal lesions. After two weeks the patient returned and informed us that the dermatologist had confirmed a diagnosis of Discoid Lupus Erythematosus. Results of the oral biopsy where as follows:
Palatal lesion – Fibrous epuli
Buccul mucosa – normal oral mucosa, mild hyperkleratosis
Alveolar ridge – normal keratinised mucosa
Discussion
Lupus erythematosus (LE) is thought to be an autoimmune disease among other connective tissue diseases, however its true aetiology is unknown. Lupus occurs in all age groups with a mean age varying from 21 years to 50 years and more often in females . Although LE is an autoimmune disease, it is thought to result from an interplay of certain genetic factors, environmental factors like ultraviolet light, and hormonal factors with antibodies.
Subtypes of lupus erythematosus (LE) includes
- chronic cutaneous LE,
- subacute cutaneous LE, and
- systemic or acute cutaneous LE.
Discoid LE lupus erythematosus (DLE), a chronic dermatological disease, is the most common form of chronic LE. Lesions may be part of systemic lupus or remain a separate diagnostic entity. Systemic lupus erythematosus (SLE) is a florid disease with systemic involvement of heart, lungs, brain, kidneys and other organs called
Lesions appear as an erythematous papule or plaque, usually on the head or neck, with an adherent scale. The lesion tends to spread centrifugally and as it progresses there is follicular plugging and pigmentary changes, generally hyperpigmentation at the periphery, and hypopigmentation with atrophy, scarring, and telengiectasia at the center of the lesion. In the localized variety of discoid lupus the lesions tend to be confined to the head and neck and in the generalized variety they occur both above and below the neck . Mucosal surfaces may be affected by lesions that appear identical to discoid lesions of the skin or by lesions that may simulate lichen planus. Palms and soles can be involved however this is rare.
Diagnosis of the cutaneous manifestations of LE is based on clinical, histopathology, and immunohistology of skin lesions. In addition, serum autoantibodies are considered immunologic markers for distinct clinical types however they are uncommon for discoid lupus. Histology of oral mucosa may be of a lichenoid tissue or appear as normal. DLE tends to run a less severe course than SLE and has a better prognosis. Hypertrophic lesions of LE must be differentiated from warts, keratoacanthomas, or squamous cell carcinoma.
Photosensitivity shows a strong association with the manifestation of all Cutaneous LE subtypes, and the abnormal reactivity to ultraviolet (UV) light is an important factor in the pathogenesis of both cutaneous and systemic disease. A potentially crucial role in the initiation of the autoimmune reaction cascade has been attributed to UV-induced keratinocyte apoptosis .
These patients are generally managed and treated by dermatologists. The most important goal is to ensure that there is no systemic involvement and exclude systemic LE as a diagnosis. Early effective treatment may lead to total clearing of skin lesions, but failure of treatment results in permanent scarring. The depressed scars, hair loss, and pigmentary changes are extremely disfiguring, particularly in darker-skinned people. Management of discoid lupus must include sun avoidance and the liberal application of sunscreens. Patients should be educated about the use of sunscreens and protective clothing. Medications used include corticosteroids and immunosuppressive drugs. The role of medical and dental professionals is to recognise and refer these patients to dermatologists so that scarring and disfigurement may be avoided.
References:
S Panjwani, Early Diagnosis and Treatment of Discoid Lupus Erythematosus; JABFM 2009 Vol. 22 No. 2
L Uva, D Miguel, C Pinheiro et al, Review Article: CutaneousManifestations of Systemic Lupus Erythematosus ; Autoimmune Diseases Vol 2012, Article ID 834291,doi:10.1155/2012/834291 Hindawi Publishing Corporation