Lupus erythematosus

Specialty| Rheumatology

Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues.[1] Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. The most common and most severe form is systemic lupus erythematosus.

## Contents

* 1 Signs and symptoms
* 1.1 Photosensitivity
* 2 Genetics
* 2.1 Age difference
* 2.2 Differences in ethnicity
* 3 Diagnosis
* 3.1 Classification
* 4 Treatment
* 5 Epidemiology
* 5.1 Worldwide
* 5.2 United Kingdom
* 5.3 United States
* 6 In popular culture
* 7 See also
* 8 References
* 9 External links

## Signs and symptoms[edit | edit source]

Symptoms vary from person to person, and may come and go. Almost everyone with lupus has joint pain and swelling. Some develop arthritis. Frequently affected joints are the fingers, hands, wrists, and knees.[citation needed] Other common symptoms include:

* chest pain during respiration
* joint pain
* painless oral ulcer
* fatigue
* weight loss
* fever with no other cause
* general discomfort, uneasiness, or ill feeling (malaise)
* hair loss
* sensitivity to sunlight
* a "butterfly" facial rash, seen in about half of people with SLE
* swollen lymph nodes[2]

* Chronic discoid lupus erythematosus

* Chronic discoid lupus erythematosus

* Chronic discoid lupus erythematosus

* Chronic discoid lupus erythematosus

### Photosensitivity[edit | edit source]

Photosensitivity is a known symptom of lupus, but its relationship to and influence on other aspects of the disease remain to be defined.[3] Causes of photosensitivity may include:

* change in autoantibody location
* cytotoxicity
* induction of apoptosis with autoantigens in apoptotic blebs
* upregulation of adhesion molecules and cytokines
* induction of nitric oxide synthase expression
* ultraviolet-generated antigenic DNA
* tumor necrosis factor alpha

## Genetics[edit | edit source]

It is typically believed that lupus is influenced by multiple genes. Lupus is usually influenced by gene polymorphisms, 30 of which have now been linked with the disorder. Some of these polymorphisms have been linked very tentatively, however, as the role that they play or the degree to which they influence the disease is unknown. Other genes that are commonly thought to be associated with lupus are those in the human leukocyte antigen (HLA) family. There have been several cases wherein a single gene influence appears to be present, but this is rare. When a single gene deficiency does cause lupus, it is usually attributed to the complement protein genes C1, C2, or C4. The influence of sex chromosomes and environmental factors are also noteworthy. Usually, these factors contribute to lupus by influencing the immune system.[4] Several studies also indicate a potential association of lupus with mutations in DNA repair genes. [5]

### Age difference[edit | edit source]

Lupus can develop in people at any age, but it does most commonly at ages 15 to 44, with varying results. Typically, the manifestation of the disease tends to be more acute in those of younger age. Women are more likely to get it than men. Patients with juvenile-onset lupus are more vulnerable to mucocutaneous manifestations of the disease (alopecia, skin rash, and ulceration of the mucus membranes) than any other age group, and they are also more susceptible to evaluation of pulmonary artery pressure.[6] However, patients with late-onset lupus have a much higher mortality rate. Nearly 50% of those with late-onset lupus die of their affliction. Women who are of childbearing age are also particularly at risk.[7]

### Differences in ethnicity[edit | edit source]

Substantial data have been found to indicate that certain ethnic populations could be more at risk for lupus erythematosus and to have a better or worse prognosis. Asian, African, and Native Americans are more likely to get lupus than Caucasians. Caucasians seem generally to have a milder manifestation of the disease. Their survival rates after five years were typically around 94–96%, while patients of African and some Asian ethnicities had survival rates closer to 79–92%. The only documented ethnic group that had a higher survival rate than Caucasians was Koreans, who had survival rates nearer to 98%. Among Caucasians, the most common causes of death were complications involving the cardiovascular system, the respiratory system, and malignancies.[8][9] Atherosclerotic cardiovascular disease is more prevalent in African Americans with lupus than in Caucasians with lupus.[10]

## Diagnosis[edit | edit source]

For the diagnosis of lupus, 4 out of 11 signs must be present.[11]

Testing may include:

* Antinuclear antibody (ANA)
* CBC with differential
* Chest X-ray
* Serum creatinine
* Urinalysis[11]

### Classification[edit | edit source]

Lupus erythematosus may manifest as systemic disease or in a purely cutaneous form also known as incomplete lupus erythematosus. Lupus has four main types:[citation needed]

* systemic
* discoid
* drug-induced
* neonatal

Of these, systemic lupus erythematosus (also known as SLE) is the most common and serious form.

A more thorough categorization of lupus includes the following types:[12][13]

* acute cutaneous lupus erythematosus
* subacute cutaneous lupus erythematosus
* discoid lupus erythematosus (chronic cutaneous
*       * childhood discoid lupus erythematosus
* generalized discoid lupus erythematosus
* localized discoid lupus erythematosus
* chilblain lupus erythematosus (Hutchinson)
* lupus erythematosus-lichen planus overlap syndrome
* lupus erythematosus panniculitis (lupus erythematosus profundus)
* tumid lupus erythematosus
* verrucous lupus erythematosus (hypertrophic lupus erythematosus)
* cutaneous lupus mucinosis
* complement deficiency syndromes
* drug-induced lupus erythematosus
* neonatal lupus erythematosus
* systemic lupus erythematosus

## Treatment[edit | edit source]

See also: Systemic lupus erythematosus § Treatment

Treatment consists primarily of immunosuppressive drugs (e.g., hydroxychloroquine and corticosteroids). A second-line drug is methotrexate in its low-dose schedule.[14] In 2011, the U.S. Food and Drug Administration (FDA) approved the first new drug for lupus in more than 50 years to be used in the US, belimumab.[15] In addition to medical therapy, cognitive behavioural therapy has also been demonstrated to be effective in reducing stress, anxiety, and depression due to the psychological and social impacts that lupus may have.[16]

People with SLE treated with standard care experience a higher risk of opportunistic infections and death than the general population. This risk is higher in men and in African Americans.[17]

## Epidemiology[edit | edit source]

### Worldwide[edit | edit source]

* An estimated 5 million people worldwide have some form of lupus disease.[18]
* 70% of lupus cases diagnosed are systemic lupus erythematosus.[18]
* 20% of people with lupus will have a parent or sibling who already has lupus or may develop lupus.[18]
* about 5% of the children born to individuals with lupus will develop the illness.[18]

### United Kingdom[edit | edit source]

* Females in the UK are seven times more likely to be diagnosed with SLE than males.[19]
* The estimated number of females in the UK with SLE is 21,700, and the number of males is 3000 — a total of 24,700, or 0.041% of the population.[19]
* SLE is more common amongst certain ethnic groups than others, especially those of African origin.[19]

### United States[edit | edit source]

* Lupus occurs from infancy to old age, with peak occurrence between ages 15 and 40.[15]
* Lupus affects females in the US 6 to 10 times more often than males.[15]
* Prevalence data are limited. Estimates vary and range from 1.8 to 7.6 cases per 100,000 persons per year in parts of the continental United States.[15]

## In popular culture[edit | edit source]

* In the early seasons of the television show House, members of the eponymous character's medical team often suggested lupus as a diagnosis for their patients, only to be rebuked. The rarity of legitimate lupus diagnoses in the show eventually became a running gag.[citation needed]

## See also[edit | edit source]

* List of cutaneous conditions
* List of target antigens in pemphigoid
* List of immunofluorescence findings for autoimmune bullous conditions
* List of human leukocyte antigen alleles associated with cutaneous conditions
* List of people with lupus

## References[edit | edit source]

1. ↑ Fitzpatrick, Thomas B.; Klauss Wolff; Wolff, Klaus Dieter; Johnson, Richard R.; Suurmond, Dick; Richard Suurmond (2005). Fitzpatrick's color atlas and synopsis of clinical dermatology. New York: McGraw-Hill Medical Pub. Division. ISBN 978-0-07-144019-6.[page needed]
2. ↑ "Systemic Lupus Erythematosus (Lupus, SLE)". PubMed Health. National Library of Medicine. Archived from the original on 2014-02-28. Retrieved 2018-05-13.
3. ↑ Scheinfeld, Noah; Deleo, Vincent A (2004). "Photosensitivity in lupus erythematosus". Photodermatology, Photoimmunology and Photomedicine. 20 (5): 272–9. doi:10.1111/j.1600-0781.2004.00094.x. PMID 15379880. S2CID 25740605.
4. ↑ Kiriakidou, Marianthi; Cotton, D; Taichman, D; Williams, S (2013). "Systemic Lupus Erythematosus". Annals of Internal Medicine. 159 (7): ITC4–1. CiteSeerX 10.1.1.1008.5428. doi:10.7326/0003-4819-159-7-201310010-01004. PMID 24081299.
5. ↑ Meas R, Burak MJ, Sweasy JB (August 2017). "DNA repair and systemic lupus erythematosus". DNA Repair (Amst.). 56: 174–182. doi:10.1016/j.dnarep.2017.06.020. PMC 5543809. PMID 28623091.
6. ↑ Adrovic, Amra; Dedeoglu, Reyhan; Sahin, Sezgin; Barut, Kenan; Koka, Aida; Cengiz, Dicle; Oztunc, Funda; Kasapcopur, Ozgur (2018-02-20). "Evaluation of pulmonary artery pressure in patients with juvenile systemic lupus erythematosus (jSLE)". Bosnian Journal of Basic Medical Sciences. 18 (1): 66–71. doi:10.17305/bjbms.2017.2178. ISSN 1840-4812. PMC 5826676. PMID 28704174. Archived from the original on 2020-11-16. Retrieved 2020-11-12.
7. ↑ Feng, X.; Zou, Y.; Pan, W.; Wang, X.; Wu, M.; Zhang, M.; Tao, J.; Zhang, Y.; Tan, K.; Li, J.; Chen, Z.; Ding, X.; Qian, X.; Da, Z.; Wang, M.; Sun, L. (2013). "Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus". Lupus. 23 (3): 327–34. doi:10.1177/0961203313513508. PMID 24297642. S2CID 11694732.
8. ↑ Voss, A.; Laustrup, H.; Hjelmborg, J.; Junker, P. (2013). "Survival in systemic lupus erythematosus, 1995–2010. A prospective study in a Danish community". Lupus. 22 (11): 1185–91. doi:10.1177/0961203313498796. PMID 23873432. S2CID 46247629.
9. ↑ "Lupus Facts, Symptoms, Rash, Effects, Complications, and More." WebMD, n.d. Web. 6 October 2014. http://www.webmd.com/lupus/arthritis-lupus Archived 2014-10-11 at the Wayback Machine.
10. ↑ Alenghat, Francis J (2016). "The Prevalence of Atherosclerosis in Those with Inflammatory Connective Tissue Disease by Race, Age, and Traditional Risk Factors". Scientific Reports. 6: 20303. Bibcode:2016NatSR...620303A. doi:10.1038/srep20303. PMC 4740809. PMID 26842423.
11. ↑ 11.0 11.1 "Systemic lupus erythematosus: MedlinePlus Medical Encyclopedia". Medline Plus. US National Library of Medicine. Archived from the original on 2019-09-23. Retrieved 2017-11-19.
12. ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Disease of the Skin: Clinical Dermatology. (10th ed.). Saunders. Chapter 8. ISBN 0-7216-2921-0.[page needed]
13. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.[page needed]
14. ↑ Böhm I (2004). "Increased peripheral blood B-cells expressing the CD5 molecules in association tp autoantibodies in patients with lupus erythematosus and evidence to selectively down-modulate them". Biomed Pharmacother. 58 (5): 338–43. doi:10.1016/j.biopha.2004.04.010. PMID 15194170.
15. ↑ 15.0 15.1 15.2 15.3 "Lupus - Arthritis". CDC. 2018-10-18. Archived from the original on 2017-12-11. Retrieved 2017-09-09.
16. ↑ Greco, Carol M; Rudy, Thomas E; Manzi, Susan (2004). "Effects of a stress-reduction program on psychological function, pain, and physical function of systemic lupus erythematosus patients: A randomized controlled trial". Arthritis Care & Research. 51 (4): 625–34. doi:10.1002/art.20533. PMID 15334437.
17. ↑ Dospinescu, Paula; Shamliyan, Tatyana A. (2017-07-01). "Additional Improvements in Clinical Response From Adjuvant Biologic Response Modifiers in Adults With Moderate to Severe Systemic Lupus Erythematosus Despite Immunosuppressive Agents: A Systematic Review and Meta-analysis". Clinical Therapeutics. 39 (7): 1479–1506.e45. doi:10.1016/j.clinthera.2017.05.359. ISSN 0149-2918. PMID 28673504. Archived from the original on 2021-08-28. Retrieved 2019-01-17.
18. ↑ 18.0 18.1 18.2 18.3 "Media Relations". Archived from the original on June 29, 2013. Retrieved August 24, 2013.
19. ↑ 19.0 19.1 19.2 "Lupus". Arthritis Research UK. Archived from the original on February 1, 2014. Retrieved August 24, 2013.

## External links[edit | edit source]

Classification|

* ICD-10: L93 (ILDS L93.010)
* ICD-9-CM: 695.4

|

* v
* t
* e

Cutaneous keratosis, ulcer, atrophy, and necrobiosis

Epidermal thickening|

* keratoderma: Keratoderma climactericum
* Paraneoplastic keratoderma
* Acrokeratosis paraneoplastica of Bazex
* Aquagenic keratoderma
* Drug-induced keratoderma
* psoriasis
* Keratoderma blennorrhagicum

* keratosis: Seborrheic keratosis
* Clonal seborrheic keratosis
* Common seborrheic keratosis
* Irritated seborrheic keratosis
* Seborrheic keratosis with squamous atypia
* Reticulated seborrheic keratosis
* Dermatosis papulosa nigra
* Keratosis punctata of the palmar creases

* other hyperkeratosis: Acanthosis nigricans
* Confluent and reticulated papillomatosis
* Callus
* Ichthyosis acquisita
* Arsenical keratosis
* Chronic scar keratosis
* Hyperkeratosis lenticularis perstans
* Hydrocarbon keratosis
* Hyperkeratosis of the nipple and areola
* Inverted follicular keratosis
* Lichenoid keratosis
* Multiple minute digitate hyperkeratosis
* PUVA keratosis
* Reactional keratosis
* Stucco keratosis
* Thermal keratosis
* Viral keratosis
* Warty dyskeratoma
* Waxy keratosis of childhood

* other hypertrophy: Keloid
* Hypertrophic scar
* Cutis verticis gyrata

Necrobiosis/granuloma|

| Necrobiotic/palisading|

* Granuloma annulare
* Perforating
* Generalized
* Subcutaneous
* Granuloma annulare in HIV disease
* Localized granuloma annulare
* Patch-type granuloma annulare
* Necrobiosis lipoidica
* Annular elastolytic giant-cell granuloma
* Granuloma multiforme
* Necrobiotic xanthogranuloma
* Palisaded neutrophilic and granulomatous dermatitis
* Rheumatoid nodulosis
* Interstitial granulomatous dermatitis/Interstitial granulomatous drug reaction

|

Foreign body granuloma|

* Beryllium granuloma
* Mercury granuloma
* Silica granuloma
* Silicone granuloma
* Zirconium granuloma
* Soot tattoo
* Tattoo
* Carbon stain

Other/ungrouped|

* eosinophilic dermatosis
* Granuloma faciale

Dermis/
localized CTD|

| Cutaneous lupus
erythematosus|

* chronic: Discoid
* Panniculitis

* subacute: Neonatal

* ungrouped: Chilblain
* Lupus erythematosus–lichen planus overlap syndrome
* Tumid
* Verrucous
* Rowell's syndrome

|

Scleroderma/
Morphea|

* Localized scleroderma
* Localized morphea
* Morphea–lichen sclerosus et atrophicus overlap
* Generalized morphea
* Atrophoderma of Pasini and Pierini
* Pansclerotic morphea
* Morphea profunda
* Linear scleroderma

Atrophic/
atrophoderma|

* Lichen sclerosus
* Anetoderma
* Schweninger–Buzzi anetoderma
* Jadassohn–Pellizzari anetoderma
* Atrophoderma of Pasini and Pierini
* Acrodermatitis chronica atrophicans
* Semicircular lipoatrophy
* Follicular atrophoderma
* Linear atrophoderma of Moulin

Perforating|

* Kyrle disease
* Reactive perforating collagenosis
* Elastosis perforans serpiginosa
* Perforating folliculitis
* Acquired perforating dermatosis

Skin ulcer|

* Pyoderma gangrenosum

Other|

* Calcinosis cutis
* Sclerodactyly
* Poikiloderma vasculare atrophicans
* Ainhum/Pseudo-ainhum

* v
* t
* e

Urticaria and erythema

Urticaria
(acute/chronic)|

| Allergic urticaria|

* Urticarial allergic eruption

|

Physical urticaria|

* Cold urticaria
* Familial
* Primary cold contact urticaria
* Secondary cold contact urticaria
* Reflex cold urticaria

* Heat urticaria
* Localized heat contact urticaria
* Solar urticaria

* Dermatographic urticaria
* Vibratory angioedema
* Pressure urticaria
* Cholinergic urticaria
* Aquagenic urticaria

Other urticaria|

* Acquired C1 esterase inhibitor deficiency
* Adrenergic urticaria
* Exercise urticaria
* Galvanic urticaria
* Schnitzler syndrome
* Urticaria-like follicular mucinosis

Angioedema|

* Episodic angioedema with eosinophilia
* Hereditary angioedema

Erythema|

| Erythema multiforme/
drug eruption|

* Erythema multiforme minor
* Erythema multiforme major
* Stevens–Johnson syndrome, Toxic epidermal necrolysis
* panniculitis (Erythema nodosum)
* Acute generalized exanthematous pustulosis

|

Figurate erythema|

* Erythema annulare centrifugum
* Erythema marginatum
* Erythema migrans
* Erythema gyratum repens

Other erythema|

* Necrolytic migratory erythema
* Erythema toxicum
* Erythroderma
* Palmar erythema
* Generalized erythema

*[v]: View this template
*[t]: Discuss this template
*[e]: Edit this template