Discoid lupus erythematosus

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Discoid lupus erythematosus
Seal 2012.jpg
Discoid lupus erythematosus lesion on the face of musician Seal.
SpecialtyDermatology

Discoid lupus erythematosus (DLE or discoid lupus) is a chronic skin condition of sores with inflammation and scarring favouring the face, ears, and scalp and at times on other body areas. These lesions develop as a red, inflamed patch with a scaling and crusty appearance. The centre areas may appear lighter in colour with a rim darker than the normal skin.

Discoid lupus erythematosus can be divided into localised, generalised, and childhood discoid lupus erythematosus.[1]

Presentation[edit]

Discoid lupus erythematosus skin lesions first present as dull or purplish red, disc-shaped flat or raised and firm areas of skin.[1][2] These lesions then develop increasing amounts of white, adherent scale.[1][2] Finally, the lesions develop extensive scarring and/or atrophy, as well as pigment changes.[1] They may also have overlying dried fluid, known as crust.[3] On darker skin, the lesions often lose skin pigmentation in the center and develop increased, dark skin pigmentation around the rim.[1] On lighter skin, the lesions often develop a gray color or have very little color change.[1]

The skin lesions are most often in sun-exposed areas localized above the neck, with favored sites being the scalp, bridge of the nose, upper cheeks, lower lip, and ears.[1][2] 24% of patients also have lesions in the mouth (most often the palate), nose, eye, or vulva, which are all mucosal parts of the body.[1][2]

When discoid lupus is on the scalp, it starts as a red flat or raised area of skin that then loses hair and develops extensive scarring.[1] The lesions often lose skin pigment and become white with areas of increased skin pigment, with or without areas of redness, and have a sunken appearance.[1] They can have a smooth surface or have visible, dilated hair follicles on the surface.[1] When discoid lupus is on the lip, it often has a gray or red color with a thickened top layer of skin (known as hyperkeratosis), areas where the top layer has worn away (known as erosion), and a surrounding rim of redness.[1]  

More rarely, patients may have lesions on the head and neck as well as the arms and trunk.[1]

Patients may state that their lesions are itchy, tender, or asymptomatic.[2][1] In addition to their skin lesions, they may also have swelling and redness around their eyes.[1]

Complications[edit]

Darker-skinned patients are often left with severe scarring and skin color changes even after the lesions get better.[2] In addition, these patients have an increased risk for aggressive skin squamous cell carcinoma, although this is a rare complication.[1]

Causes[edit]

Sun exposure triggers lesions in people with discoid lupus erythematous.[2] Evidence does not clearly demonstrate a genetic component to DLE; however, genetics may predispose certain people to disease.[2]

Mechanism[edit]

Most experts consider DLE an autoimmune disease since pathologists see antibodies when they biopsy the lesions and look at the tissue under the microscope.[2] However, scientists do not understand the connection between these antibodies and the lesions seen in discoid lupus.[2]

Possibly, UV light damages skin cells, which then release material from their nuclei.[2] This material diffuses to the dermoepidermal junction, where it binds to circulating antibodies, thereby leading to a series of inflammatory reactions by the immune system.[2]

Alternatively, dysfunctional T cells may lead to the disease.[2]

Diagnosis[edit]

Diagnosis is confirmed through biopsy.[2] Typical biopsy findings include deposits of IgG and IgM antibodies at the dermoepidermal junction on direct immunofluorescence.[1][2] This finding is 90% sensitive; however, false positives can occur with biopsies of facial lesions.[2] In addition, pathologists often see groups of white blood cells, particularly T helper cells, around the follicles and blood vessels in the dermis.[1][2] The epidermis appears thin and has excess amounts of keratin clogging the openings of the follicles.[1][2] The basal layer of the epidermis sometimes appears to have holes in it since some of the cells in this layer have broken apart.[2]

In order to help with diagnosis, the doctor may peel off the top layer of scale from a patient's lesions in order to look at its underside.[1] If the patients does indeed have discoid lupus, the doctor may see tiny spines of keratin that look like carpet tacks and are called langue au chat.[1]

Treatment[edit]

Treatment for discoid lupus erythematosus includes smoking cessation and a sunscreen that protects against both UVA and UVB light as well as very strong topical steroids or steroids injected into the lesions.[2] If this does not help the patient, his or her physician can prescribe an antimalarial medication such as oral hydroxychloroquine or chloroquine.[2] Other oral medications used to treat discoid lupus include retinoids (isotretinoin or acitretin), dapsone, thalidomide, azathioprine, methotrexate, or gold.[2] The topical steroid fluocinonide is more effective than hydrocortisone in the treatment of discoid lupus erythematosus.[4] For oral treatment, hydroxychloroquine and acitretin are equally effective; however, acitretin was associated with more adverse effects.[4]

Prognosis[edit]

Discoid lupus erythematosus is a chronic condition, and lesions will last for several years without treatment.[3][2] 50% of patients will eventually get better on their own.[2] If a patient does not have any signs of systemic lupus erythematosus, such as generalized hair loss, ulcers in the mouth or nose, Raynaud's phenomenon, arthritis, or fever at the time that he develops discoid lupus, he will most likely only have discoid lupus and will never develop systemic lupus erythematosus.[1] [2]

Epidemiology[edit]

Discoid lupus has an unknown incidence.[2] The disease tends to affect young adults, and women are affected more than men in a 2:1 ratio.[1]

Society and culture[edit]

The musician Seal has this skin condition. Michael Jackson was also known to have had it.[5]

Other Animals[edit]

Dogs and horses can also get discoid lupus.[6][7]

See also[edit]

References[edit]

  1. ^ a b c d e f g h i j k l m n o p q r s t u v w James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.) Saunders. Chapter 8. ISBN 0-7216-2921-0.
  2. ^ a b c d e f g h i j k l m n o p q r s t u v w x y z author., Marks, James G., Jr.,. Lookingbill and Marks' principles of dermatology. ISBN 9780323430425. OCLC 1024315813.
  3. ^ a b editor., Fitzpatrick, James E., 1948- editor. Morelli, Joseph G. Dermatology secrets plus. OCLC 1010741108.
  4. ^ a b Jessop, Sue; Whitelaw, David A; Grainge, Matthew J; Jayasekera, Prativa (2017-05-05). "Drugs for discoid lupus erythematosus". Cochrane Database of Systematic Reviews. doi:10.1002/14651858.CD002954.pub3. ISSN 1465-1858.
  5. ^ Finn, Robin (June 5, 1996). "At Lunch With: Seal; From a Crucible Of Early Pain Comes the Gold Of Stardom". The New York Times.
  6. ^ Olivry, Thierry; Linder, Keith E.; Banovic, Frane (2018-04-18). "Cutaneous lupus erythematosus in dogs: a comprehensive review". BMC Veterinary Research. 14. doi:10.1186/s12917-018-1446-8. ISSN 1746-6148. PMC 5907183. PMID 29669547.
  7. ^ "Immune-Mediated Dermatoses". Veterinary Clinics of North America: Equine Practice. 29 (3): 607–613. 2013-12-01. doi:10.1016/j.cveq.2013.08.001. ISSN 0749-0739.

External links[edit]

Classification