Discoid lupus erythematosus

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Discoid lupus erythematosus
Other namesDLE, Discoid lupus
Discoid lupus etythermayouses new image.jpg
Discoid lupus erythematosus lesion on the head
Specialty Dermatology, Immunology

Discoid lupus erythematosus is the most common type of chronic cutaneous lupus (CCLE), an autoimmune skin condition on the lupus erythematosus spectrum of illnesses. [1] [2] It presents with red, painful, inflamed and coin-shaped patches of skin with a scaly and crusty appearance, most often on the scalp, cheeks, and ears. Hair loss may occur if the lesions are on the scalp. [3] The lesions can then develop severe scarring, and the centre areas may appear lighter in color with a rim darker than the normal skin. [3] These lesions can last for years without treatment. [4]

Contents

Patients with systemic lupus erythematous develop discoid lupus lesions with some frequency. [4] However, patients who present initially with discoid lupus infrequently develop systemic lupus. [3] Discoid lupus can be divided into localized, generalized, and childhood discoid lupus. [3]

The lesions are diagnosed by biopsy. [5] Patients are first treated with sunscreen and topical steroids. [5] If this does not work, an oral medication—most likely hydroxychloroquine or a related medication—can be tried. [5]

Signs and symptoms

Morphology of lesions

Discoid lupus erythematosus (DLE) skin lesions first present as dull or purplish red, disc-shaped flat or raised and firm areas of skin. [3] [5] These lesions then develop increasing amounts of white, adherent scale. [3] [5] Finally, the lesions develop extensive scarring and/or atrophy, as well as pigment changes. [3] They may also have overlying dried fluid, known as crust. [4] On darker skin, the lesions often lose skin pigmentation in the center and develop increased, dark skin pigmentation around the rim. [3] On lighter skin, the lesions often develop a gray color or have very little color change. [3] More rarely, the lesions may be bright red and look like hives. [3]

Location of lesions

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 ear and hands [3] [5] 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. [3] [5]

More rarely, patients may have lesions on the head and neck as well as the arms and trunk. [3]

Special characteristics of some lesions

Scalp lesions

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. [3] 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. [3] They can have a smooth surface or have visible, dilated hair follicles on the surface. [3]

Lip lesions

When discoid lupus is on the lip, it often has a grey or red colour 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. [3]

Other symptoms

Patients may state that their lesions are itchy, tender, or asymptomatic. [3] [5] In addition to their skin lesions, they may also have swelling and redness around their eyes, as well as blepharitis. [3] [6]

Complications

Darker-skinned patients are often left with severe scarring and skin color changes even after the lesions get better. [5] In addition, these patients have an increased, though still small, risk for aggressive skin squamous cell carcinoma. [3]

Causes

Sun exposure triggers lesions in people with discoid lupus erythematous (DLE). [5] Evidence does not clearly demonstrate a genetic component to DLE; however, genetics may predispose certain people to disease. [5]

Mechanism

Most experts consider DLE an autoimmune disease since pathologists see antibodies when they biopsy the lesions and look at the tissue under the microscope. [5] However, scientists do not understand the connection between these antibodies and the lesions seen in discoid lupus. [5]

Possibly, UV light damages skin cells, which then release material from their nuclei. [5] 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. [5]

Alternatively, dysfunctional T cells may lead to the disease. [5]

Diagnosis

When a patient initially presents with discoid lupus, the doctor should ensure that the patient does not have systemic lupus erythematosus. [5] The doctor will order tests to check for anti-nuclear antibodies in the patient's serum, low white blood cell levels, and protein and/or blood in the urine. [3] [5]

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. [3] If the patients do indeed have discoid lupus, the doctor may see tiny spines of keratin that look like carpet tacks and are called langue au chat. [3]

Diagnosis is confirmed through biopsy. [5] Typical biopsy findings include deposits of IgG and IgM antibodies at the dermoepidermal junction on direct immunofluorescence. [3] [5] This finding is 90% sensitive; however, false positives can occur with biopsies of facial lesions. [5] In addition, pathologists often see groups of white blood cells, particularly T helper cells, around the follicles and blood vessels in the dermis. [3] [5] The epidermis appears thin and has effaced rete ridges as well as excess amounts of keratin clogging the openings of the follicles. [3] [5] The basal layer of the epidermis sometimes appears to have holes in it since some of the cells in this layer have broken apart. [5] The remains of skin cells that have died through a process called apoptosis are visible in the upper layer of the dermis and the basal layer of the epidermis. [3]

The differential diagnosis includes actinic keratoses, sebborheic dermatitis, lupus vulgaris, sarcoidosis, drug rash, Bowen's disease, lichen planus, tertiary syphilis, polymorphous light eruption, lymphocytic infiltration, psoriasis, and systemic lupus erythematosus. [3] [5]

Classification

Discoid lupus can be broadly classified into localized discoid lupus and generalized discoid lupus based on the location of the lesions. [3] Patients who develop discoid lupus in childhood also have their own sub-type of disease. [3]

Hypertrophic lupus and lupus profundus are two special types of discoid lupus distinguished by their characteristic morphological findings. [4]

Finally, many patients with systemic lupus also develop discoid lupus lesions. [4]

Localized

Most people with discoid lupus only have lesions above the neck and therefore have localized discoid lupus erythematosus. [3]

Generalized

Rarely, patients may have lesions above and below the neck; these patients have generalized discoid lupus erythematosus. [3] [5] In addition to lesions in the typical above-the-neck locations, patients with generalized discoid lupus often have lesions on the thorax and the arms. [3] These patients are often bald, with abnormal skin pigment on their scalp, and have severe scarring of the face and arms. [3] Patients with generalized discoid lupus often have abnormal lab tests, such as an elevated ESR or a low white blood cell count. [3] They also often have auto-antibodies, such as ANA or anti-ssDNA antibody. [3]

Childhood

When patients develop discoid lupus in childhood, it differs from typical discoid lupus in several ways. Boys and girls are equally affected, and these patients later develop SLE more often. [3] These patients also typically do not have any abnormal sensitivity to the sun. [3]

Special types of discoid lupus lesions

Hypertrophic lupus

Some experts consider hypertrophic lupus erythematosus—which consists of lesions covered by a very thick, keratin-filled scale—an unusual subset of discoid lupus. [4] Others consider it a distinct entity. [3]

Lupus profundus

If a patient has discoid lupus lesions on top of lupus panniculitis, they have lupus profundus. [4] These patients have firm, nontender nodules with defined borders underneath their discoid lupus lesions. [3]

Systemic lupus erythematosus with discoid lupus lesions

In general, patients with discoid lupus who have only skin disease and no systemic symptoms have a genetically distinct disease from patients with SLE. [5] However, 25% of patients with SLE get discoid lupus lesions at some point as part of their disease. [4]

Treatment

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. [5] Other topical treatments, tacrolimus or pimecrolimus can also be used. [2] [7] If this does not help the patient, his or her physician can prescribe an antimalarial medication such as oral hydroxychloroquine or chloroquine. [5] Other oral medications used to treat discoid lupus include retinoids (isotretinoin or acitretin), dapsone, thalidomide (teratogenic, side effects include peripheral neuropathy), azathioprine, methotrexate, or gold. [2] [5] The topical steroid fluocinonide is more effective than hydrocortisone in the treatment of discoid lupus erythematosus. [8] For oral treatment, hydroxychloroquine and acitretin are equally effective; however, acitretin was associated with more adverse effects. [8]

Pulsed dye laser is also an effective treatment for patients with localized discoid lupus. [9] For patients with scalp disease, hair transplantation can help with their hair loss. [10]

Prognosis

Discoid lupus erythematosus is a chronic condition, and lesions will last for several years without treatment. [4] [5] 50% of patients will eventually get better on their own. [5] 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 they develop discoid lupus, they will most likely only have discoid lupus and will never develop systemic lupus erythematosus. [3] [5]

Epidemiology

Discoid lupus has an unknown incidence, although it is two to three times more common than systemic lupus erythematosus. [5] [6] The disease tends to affect young adults, and women are affected more than men in a 2:1 ratio. [3]

Society and culture

The musician Seal has this skin condition. [11]

Singer Michael Jackson was reportedly diagnosed with discoid lupus in 1984; the condition might have damaged his nasal cartilage and led to some of his cosmetic surgery. [12]

In animals

A dog with a lesion on the nose Canine discoid lupus.jpg
A dog with a lesion on the nose

Dogs and horses can also get discoid lupus. [13] [14]

See also

Related Research Articles

<span class="mw-page-title-main">Lichen planus</span> Human chronic inflammatory disease

Lichen planus (LP) is a chronic inflammatory and autoimmune disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, but is named for its appearance. It is characterized by polygonal, flat-topped, violaceous papules and plaques with overlying, reticulated, fine white scale, commonly affecting dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa. The hue may be gray-brown in people with darker skin. Although there is a broad clinical range of LP manifestations, the skin and oral cavity remain as the major sites of involvement. The cause is unknown, but it is thought to be the result of an autoimmune process with an unknown initial trigger. There is no cure, but many different medications and procedures have been used in efforts to control the symptoms.

<span class="mw-page-title-main">Actinic keratosis</span> Rough skin induced by UV exposure

Actinic keratosis (AK), sometimes called solar keratosis or senile keratosis, is a pre-cancerous area of thick, scaly, or crusty skin. Actinic keratosis is a disorder of epidermal keratinocytes that is induced by ultraviolet (UV) light exposure.

<span class="mw-page-title-main">Mycosis fungoides</span> Most common form of cutaneous T-cell lymphoma

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<span class="mw-page-title-main">Lupus vulgaris</span> Painful facial skin nodules due to infection by M. tuberculosis

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Discoid lupus erythematosus (DLE) is an uncommon autoimmune disease of the basal cell layer of the skin. It occurs in humans and cats, more frequently occurring in dogs. It was first described in dogs by Griffin and colleagues in 1979. DLE is one form of cutaneous lupus erythematosus (CLE). DLE occurs in dogs in two forms: a classical facial predominant form or generalized with other areas of the body affected. Other non-discoid variants of CLE include vesicular CLE, exfoliative CLE and mucocutaneous CLE. It does not progress to systemic lupus erythematosus (SLE) in dogs. SLE can also have skin symptoms, but it appears that the two are either separate diseases. DLE in dogs differs from SLE in humans in that plasma cells predominate histologically instead of T lymphocytes. Because worsening of symptoms occurs with increased ultraviolet light exposure, sun exposure most likely plays a role in DLE, although certain breeds (see below) are predisposed. After pemphigus foliaceus, DLE is the second most common autoimmune skin disease in dogs.

<span class="mw-page-title-main">Pemphigoid</span> Autoimmune blistering diseases

Pemphigoid is a group of rare autoimmune blistering diseases of the skin and mucous membranes. As its name indicates, pemphigoid is similar in general appearance to pemphigus, however unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.

<span class="mw-page-title-main">Lupus erythematosus</span> Collection of human autoimmune diseases

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Pemphigus foliaceus is an autoimmune blistering disease of the skin. Pemphigus foliaceus causes a characteristic inflammatory attack at the subcorneal layer of epidermis, which results in skin lesions that are scaly or crusted erosions with an erythematous (red) base. Mucosal involvement is absent even with widespread disease.

Chilblain lupus erythematosus was initially described by Hutchinson in 1888 as an uncommon manifestation of chronic cutaneous lupus erythematosus. Chilblain lupus erythematosus is characterized by a rash that primarily affects acral surfaces that are frequently exposed to cold temperatures, such as the toes, fingers, ears, and nose. The rash is defined by oedematous skin, nodules, and tender plaques with a purple discoloration.

Tumid lupus erythematosus is a rare, but distinctive entity in which patients present with edematous erythematous plaque.

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Lupus erythematosus panniculitis presents with subcutaneous nodules that are commonly firm, sharply defined and nontender.

<span class="mw-page-title-main">Lupus</span> Autoimmune disease in which the immune system attacks healthy tissue

Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired, and a red rash which is most commonly on the face. Often there are periods of illness, called flares, and periods of remission during which there are few symptoms.

<span class="mw-page-title-main">Fungal folliculitis</span> Inflammation of hair follicles due to fungal infection

Majocchi's granuloma is a skin condition characterized by deep, pustular plaques, and is a form of tinea corporis. It is a localized form of fungal folliculitis. Lesions often have a pink and scaly central component with pustules or folliculocentric papules at the periphery. The name comes from Domenico Majocchi, who discovered the disorder in 1883. Majocchi was a professor of dermatology at the University of Parma and later the University of Bologna. This disease is most commonly caused by filamentous fungi in the genus Trichophyton.

Actinic granuloma (AG) was first described by O'Brien in 1975 as a rare granulomatous disease. Lesions appear on sun-exposed areas, usually on the face, neck, and scalp, with a slight preference for middle-aged women. They are typically asymptomatic, single or multiple, annular or polycyclic lesions measuring up to 6 cm in diameter, with slow centrifugal expansion, an erythematous elevated edge, and a hypopigmented, atrophic center.

Acute cutaneous lupus erythematosus is a cutaneous condition characterized by a bilateral malar rash and lesions that tend to be transient, and that follow sun exposure. The acute form is distinct from chronic and subacute cutaneous lupus erythematosus, which may have different types of skin lesions. Cutaneous lupus erythematosus is associated with both lupus erythematosus-specific lesions and cutaneous manifestations that are not specific to lupus erythematosus, such as oral ulcers and urticaria. Because of the diagnostic criteria used to diagnose systemic lupus erythematosus, a patient with only cutaneous manifestations may be diagnosed with the systemic form of the disease.

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References

  1. ↑ "Cutaneous Lupus Erythematosus | American Skin Association". www.americanskin.org. Retrieved 2018-12-11.
  2. 1 2 3 Wollina, Uwe; Hansel, Gesina; Koch, André; Abdel-Naser, Mohamed Badawy (2006). "Topical pimecrolimus for skin disease other than atopic dermatitis". Expert Opinion on Pharmacotherapy. 7 (14): 1967–1975. doi:10.1517/14656566.7.14.1967. PMID   17020422. S2CID   44901447.
  3. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.) Saunders. Chapter 8. ISBN   0-7216-2921-0.
  4. 1 2 3 4 5 6 7 8 9 Fitzpatrick, James E.; Morelli, Joseph G. Dermatology secrets plus. ISBN   978-0-323-31355-1. OCLC   1010741108.
  5. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 Marks, James G.; Miller, Jeffrey J.; Lookingbill, Donald P. (2019). Lookingbill and Marks' principles of dermatology. Saunders Elsevier. ISBN   978-0-323-43042-5. OCLC   1024315813.
  6. 1 2 Malagola, R.; Abicca, I.; Abbouda, A.; Arrico, L. (2015). "Ocular Complications in Cutaneous Lupus Erythematosus: A Systematic Review with a Meta-Analysis of Reported Cases". Journal of Ophthalmology. 2015: 254260. doi: 10.1155/2015/254260 . PMC   4480931 . PMID   26171240.
  7. ↑ Kouvelas, Dimitrios; Tzellos, Thrasivoulos George (2008-04-01). "Topical tacrolimus and pimecrolimus in the treatment of cutaneous lupus erythematosus: an evidence-based evaluation". European Journal of Clinical Pharmacology. 64 (4): 337–341. doi:10.1007/s00228-007-0421-2. ISSN   1432-1041. PMID   18157526. S2CID   5788593.
  8. 1 2 Jessop, Sue; Whitelaw, David A; Grainge, Matthew J; Jayasekera, Prativa (2017-05-05). "Drugs for discoid lupus erythematosus". Cochrane Database of Systematic Reviews. 2017 (5): CD002954. doi:10.1002/14651858.CD002954.pub3. ISSN   1465-1858. PMC   6481466 . PMID   28476075.
  9. ↑ Erceg, Angelina; De Jong, Elke M.J.G.; Van De Kerkhof, Peter C.M.; Seyger, Marieke M.B. (2013). "The efficacy of pulsed dye laser treatment for inflammatory skin diseases: A systematic review". Journal of the American Academy of Dermatology. 69 (4): 609–615.e8. doi:10.1016/j.jaad.2013.03.029. PMID   23711766 . Retrieved 2018-12-14.
  10. ↑ Ekelem, Chloe; Pham, Christine; Atanaskova Mesinkovska, Natasha (2018-09-05). "A Systematic Review of the Outcome of Hair Transplantation in Primary Scarring Alopecia". Skin Appendage Disorders. 5 (2): 65–71. doi:10.1159/000492539. ISSN   2296-9195. PMC   6388556 . PMID   30815438.
  11. ↑ Finn, Robin (June 5, 1996). "At Lunch With: Seal; From a Crucible Of Early Pain Comes the Gold Of Stardom". The New York Times.
  12. ↑ Alyssa Rosenberg (Feb. 2, 2016). Opinion:To understand Michael Jackson and his skin, you have to go beyond race, The Washington Post, 30 May 2020
  13. ↑ Olivry, Thierry; Linder, Keith E.; Banovic, Frane (2018-04-18). "Cutaneous lupus erythematosus in dogs: a comprehensive review". BMC Veterinary Research. 14 (1): 132. doi: 10.1186/s12917-018-1446-8 . ISSN   1746-6148. PMC   5907183 . PMID   29669547.
  14. ↑ Rosenkrantz, Wayne (2013-12-01). "Immune-Mediated Dermatoses". Veterinary Clinics of North America: Equine Practice. 29 (3): 607–613. doi:10.1016/j.cveq.2013.08.001. ISSN   0749-0739. PMID   24267678.
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