Epidemiology<\/a><\/h3><\/p>\n\n\n\n- Prevalence of DLE ranges from 9-70 cases per 100,000 population. <\/li>
- DLE incidence is 3-5 times higher in Asian and Black ethnic groups compared to White ethnic groups <\/li>
- Predominantly affects women with a female to male ratio of 3:1. <\/li>
- Most often develops in persons aged 20-40 years. <\/li>
- Oral lesions are present in 20% of DLE patients. <\/li>
- Occurrence of oral lesions in DLE patients without cutaneous involvement is rare and reported in 10% of cases <\/li><\/ul>\n\n\n\n
<\/div><\/div>\n\n\n\n
Clinical presentation<\/a><\/h3><\/p>\n\n\n\n- Most common presentation of chronic cutaneous lupus is DLE which may occur as localized type (80%) with lesions presenting on the face, ears and scalp. <\/li>
- Disseminated DLE (20% of cases) presents with lesions above and below the neck and is associated with an increased risk of progression to systemic lupus erythematosus <\/li><\/ul>\n\n\n\n
Cutaneous Lesions:<\/strong><\/em><\/p>\n\n\n\n- DLE cutaneous lesions present on sun exposed areas of the face and neck with annular erythema and follicular hyperkeratosis. A characteristic plaque over the malar area of the face and bridge of the nose in a butterfly pattern had been described. <\/li>
- The skin lesions may be accompanied by an itching or burning sensation. <\/li>
- As the skin lesions progress, central atrophy, scarring, telangiectasia and hypopigmentation occur. Irreversible scarring alopecia on the scalp also occurs <\/li><\/ul>\n\n\n\n
Oral Lesions:<\/strong><\/em><\/p>\n\n\n\n- Oral lesions occur in approximately 20% of cases and typically affects the lips, hard palate and buccal mucosa <\/li>
- Oral DLE lesions are characterized by the presence of central erythema or ulceration surrounded by hyperkeratotic papules or radiating striae, and peripheral telangiectasia (Figures 1 and 2). The \u201choneycomb\u201d appearance occurs in long-standing lesions. <\/li>
- Mucosal lesions may occur without involvement of the skin or prior to the development of skin lesions. Lip lesions may spread to involve the adjacent skin, obscuring the vermilion border. <\/li>
- Desquamative gingivitis affecting lower and\/or upper gingiva may also be present <\/li>
- With healing, erosive lesions may leave post-inflammatory pigmentation. <\/li>
- The most common symptoms of DLE include a burning sensation, photosensitivity, dryness, tenderness and pain, but lesions can be asymptomatic <\/li><\/ul>\n\n\n\n
![\"Figure](\"https:\/\/opmdcare.com\/wp-content\/uploads\/dle-image1.jpeg\")
<\/a>Figure 1: Lip lesion in DLE presenting with central atrophy surrounded by radiating hyperkeratotic striae (courtesy of Professor Ivan Alajbeg)<\/figcaption><\/figure>\n\n\n\n![\"Figure](\"https:\/\/opmdcare.com\/wp-content\/uploads\/dle-image2_compressed-1024x768.png\" "\\"Figure")
Figure 2: Oral mucosal lesion of DLE presenting with central atrophy and erosion surrounded by radiating hyperkeratotic striae (courtesy of Professor Ivan Alajbeg)<\/figcaption><\/figure>\n\n\n\n- DLE patients have increased risk of cancer, including non-melanoma skin cancer and oral cancer, compared to the general population <\/li>
- The World Health Organization categorized DLE as an oral potentially malignant disorder though malignant transformation is rare <\/li>
- More commonly, malignant transformation occurs in DLE lesions localized to the vermilion border of the lip, with the lower lip more often affected. <\/li>
- Long-term ultraviolet light exposure, chronic scarring, HPV infection and long-term immunosuppressive therapy may be predisposing factors for the development of SCC. The mean duration from the onset of DLE to the development of lip cancer is shorter compared to cancers originating from DLE lesions at other sites (10-13 years vs. 19-26 years). <\/li>
- DLE-related cancers are more aggressive and have higher metastatic potential (10-25%), recurrence rates (27-29%) and mortality (19.4%) compared to non-DLE related cancers (20%, 0.5-6% and 1%, respectively) <\/li><\/ul>\n\n\n\n
<\/div><\/div>\n\n\n\n
Differential diagnoses<\/a><\/h3><\/p>\n\n\n\nThe differential diagnosis of DLE oral lesions includes oral lichen planus (OLP), lichenoid reactions, oral leukoplakia and actinic cheilitis (when the lower lip is affected). <\/p>\n\n\n\n
In the case of OLP, lesions are more widespread and more symmetrically distributed, and the reticular pattern is more pronounced unlike in DLE oral lesions. <\/p>\n\n\n\n
Lichenoid reactions present as white striae in sites directly in contact with amalgam restoration. Following removal of the restoration, the lesion improves or resolves. <\/p>\n\n\n\n
Oral leukoplakia does not exhibit a radial pattern of hyperkeratotic striae and does not present with central atrophy. <\/p>\n\n\n\n
Actinic cheilitis usually involves the lower lip presenting with scabbing and without striae formation.<\/p>\n\n\n\n
<\/div><\/div>\n\n\n\n
Diagnosis<\/a><\/h3><\/p>\n\n\n\n- Diagnosis of DLE can be challenging in light of the similarities with oral lichen planus in histopathology. <\/li>
- Histological findings of oral DLE include hyperkeratosis with keratotic plugs, atrophy of the rete ridges, hydropic degeneration of the basal cell layer, interface mucositis with superficial or deep perivascular lymphocytic infiltrate, oedema in the lamina propria and PAS positive thickening of blood vessel walls (Figure 3). <\/li><\/ul>\n\n\n\n
![\"Figure](\"https:\/\/opmdcare.com\/wp-content\/uploads\/dle-image3-1024x643.jpeg\")
Figure 3: Histology of DLE presenting with hyperkeratotic, acanthotic stratified squamous epithelium, juxtaepithelial cell-free zone and vasodilation and oedema in the connective tissue (H and E stain, 450\u00d7) (image reproduced from Lallas et al, 2013 11).<\/figcaption><\/figure>\n\n\n\n- Direct immunofluorescence (DIF) may be helpful for further analysis. DLE lesions demonstrate linear or granular deposition of IgM, IgG and complement 3 (C3) at the basement membrane zone- the \u2018lupus band\u2019 (Figure 4). <\/li>
- In contrast to DLE, fibrinogen deposits can be found in 90-100% of the OLP patients along the basement membrane. Due to the marked difference in DIF findings between DLE and oral lichen planus, many authors believe DIF should be part of the diagnostic standard when it is suspected.<\/li><\/ul>\n\n\n\n
![\"Figure](\"https:\/\/opmdcare.com\/wp-content\/uploads\/dle-image4.jpeg\")
Figure 4: Direct immunofluorescence (DIF) of DLE showing deposit of IgM in a linear granular pattern at basement membrane zone (lupus band) (Image reproduced from Bhushan et al, 2017 (28)).<\/figcaption><\/figure>\n\n\n\n- Serological and haematological abnormalities may be detected in DLE patients. In some cases, the erythrocyte sedimentation rate may be elevated. Approximately 20% of patients have positive antinuclear antibody (ANA) and up to 20% have SS-A autoantibodies. Anti-Sm autoantibodies, usually seen in systemic lupus erythematosus, may occur in 5-20% of DLE patients.<\/li><\/ul>\n\n\n\n
<\/div><\/div>\n\n\n\n
- Prevalence of DLE ranges from 9-70 cases per 100,000 population. <\/li>
- DLE incidence is 3-5 times higher in Asian and Black ethnic groups compared to White ethnic groups <\/li>
- Predominantly affects women with a female to male ratio of 3:1. <\/li>
- Most often develops in persons aged 20-40 years. <\/li>
- Oral lesions are present in 20% of DLE patients. <\/li>
- Occurrence of oral lesions in DLE patients without cutaneous involvement is rare and reported in 10% of cases <\/li><\/ul>\n\n\n\n
<\/div><\/div>\n\n\n\n
Clinical presentation<\/a><\/h3>
<\/p>\n\n\n\n- Most common presentation of chronic cutaneous lupus is DLE which may occur as localized type (80%) with lesions presenting on the face, ears and scalp. <\/li>
- Disseminated DLE (20% of cases) presents with lesions above and below the neck and is associated with an increased risk of progression to systemic lupus erythematosus <\/li><\/ul>\n\n\n\n
Cutaneous Lesions:<\/strong><\/em><\/p>\n\n\n\n
- DLE cutaneous lesions present on sun exposed areas of the face and neck with annular erythema and follicular hyperkeratosis. A characteristic plaque over the malar area of the face and bridge of the nose in a butterfly pattern had been described. <\/li>
- The skin lesions may be accompanied by an itching or burning sensation. <\/li>
- As the skin lesions progress, central atrophy, scarring, telangiectasia and hypopigmentation occur. Irreversible scarring alopecia on the scalp also occurs <\/li><\/ul>\n\n\n\n
Oral Lesions:<\/strong><\/em><\/p>\n\n\n\n
- Oral lesions occur in approximately 20% of cases and typically affects the lips, hard palate and buccal mucosa <\/li>
- Oral DLE lesions are characterized by the presence of central erythema or ulceration surrounded by hyperkeratotic papules or radiating striae, and peripheral telangiectasia (Figures 1 and 2). The \u201choneycomb\u201d appearance occurs in long-standing lesions. <\/li>
- Mucosal lesions may occur without involvement of the skin or prior to the development of skin lesions. Lip lesions may spread to involve the adjacent skin, obscuring the vermilion border. <\/li>
- Desquamative gingivitis affecting lower and\/or upper gingiva may also be present <\/li>
- With healing, erosive lesions may leave post-inflammatory pigmentation. <\/li>
- The most common symptoms of DLE include a burning sensation, photosensitivity, dryness, tenderness and pain, but lesions can be asymptomatic <\/li><\/ul>\n\n\n\n<\/a>
Figure 1: Lip lesion in DLE presenting with central atrophy surrounded by radiating hyperkeratotic striae (courtesy of Professor Ivan Alajbeg)<\/figcaption><\/figure>\n\n\n\n Figure 2: Oral mucosal lesion of DLE presenting with central atrophy and erosion surrounded by radiating hyperkeratotic striae (courtesy of Professor Ivan Alajbeg)<\/figcaption><\/figure>\n\n\n\n - DLE patients have increased risk of cancer, including non-melanoma skin cancer and oral cancer, compared to the general population <\/li>
- The World Health Organization categorized DLE as an oral potentially malignant disorder though malignant transformation is rare <\/li>
- More commonly, malignant transformation occurs in DLE lesions localized to the vermilion border of the lip, with the lower lip more often affected. <\/li>
- Long-term ultraviolet light exposure, chronic scarring, HPV infection and long-term immunosuppressive therapy may be predisposing factors for the development of SCC. The mean duration from the onset of DLE to the development of lip cancer is shorter compared to cancers originating from DLE lesions at other sites (10-13 years vs. 19-26 years). <\/li>
- DLE-related cancers are more aggressive and have higher metastatic potential (10-25%), recurrence rates (27-29%) and mortality (19.4%) compared to non-DLE related cancers (20%, 0.5-6% and 1%, respectively) <\/li><\/ul>\n\n\n\n
<\/div><\/div>\n\n\n\n
Differential diagnoses<\/a><\/h3>
<\/p>\n\n\n\nThe differential diagnosis of DLE oral lesions includes oral lichen planus (OLP), lichenoid reactions, oral leukoplakia and actinic cheilitis (when the lower lip is affected). <\/p>\n\n\n\n
In the case of OLP, lesions are more widespread and more symmetrically distributed, and the reticular pattern is more pronounced unlike in DLE oral lesions. <\/p>\n\n\n\n
Lichenoid reactions present as white striae in sites directly in contact with amalgam restoration. Following removal of the restoration, the lesion improves or resolves. <\/p>\n\n\n\n
Oral leukoplakia does not exhibit a radial pattern of hyperkeratotic striae and does not present with central atrophy. <\/p>\n\n\n\n
Actinic cheilitis usually involves the lower lip presenting with scabbing and without striae formation.<\/p>\n\n\n\n
<\/div><\/div>\n\n\n\n
Diagnosis<\/a><\/h3>
<\/p>\n\n\n\n- Diagnosis of DLE can be challenging in light of the similarities with oral lichen planus in histopathology. <\/li>
- Histological findings of oral DLE include hyperkeratosis with keratotic plugs, atrophy of the rete ridges, hydropic degeneration of the basal cell layer, interface mucositis with superficial or deep perivascular lymphocytic infiltrate, oedema in the lamina propria and PAS positive thickening of blood vessel walls (Figure 3). <\/li><\/ul>\n\n\n\n
Figure 3: Histology of DLE presenting with hyperkeratotic, acanthotic stratified squamous epithelium, juxtaepithelial cell-free zone and vasodilation and oedema in the connective tissue (H and E stain, 450\u00d7) (image reproduced from Lallas et al, 2013 11).<\/figcaption><\/figure>\n\n\n\n - Direct immunofluorescence (DIF) may be helpful for further analysis. DLE lesions demonstrate linear or granular deposition of IgM, IgG and complement 3 (C3) at the basement membrane zone- the \u2018lupus band\u2019 (Figure 4). <\/li>
- In contrast to DLE, fibrinogen deposits can be found in 90-100% of the OLP patients along the basement membrane. Due to the marked difference in DIF findings between DLE and oral lichen planus, many authors believe DIF should be part of the diagnostic standard when it is suspected.<\/li><\/ul>\n\n\n\n
Figure 4: Direct immunofluorescence (DIF) of DLE showing deposit of IgM in a linear granular pattern at basement membrane zone (lupus band) (Image reproduced from Bhushan et al, 2017 (28)).<\/figcaption><\/figure>\n\n\n\n - Serological and haematological abnormalities may be detected in DLE patients. In some cases, the erythrocyte sedimentation rate may be elevated. Approximately 20% of patients have positive antinuclear antibody (ANA) and up to 20% have SS-A autoantibodies. Anti-Sm autoantibodies, usually seen in systemic lupus erythematosus, may occur in 5-20% of DLE patients.<\/li><\/ul>\n\n\n\n
<\/div><\/div>\n\n\n\n
- Serological and haematological abnormalities may be detected in DLE patients. In some cases, the erythrocyte sedimentation rate may be elevated. Approximately 20% of patients have positive antinuclear antibody (ANA) and up to 20% have SS-A autoantibodies. Anti-Sm autoantibodies, usually seen in systemic lupus erythematosus, may occur in 5-20% of DLE patients.<\/li><\/ul>\n\n\n\n