関: subacute cutaneous lupus erythematosus

WordNet

relating to or existing on or affecting the skin; "cutaneous nerves"; "a cutaneous infection" (同)cutaneal, dermal
any of several forms of ulcerative skin disease
a constellation in the southern hemisphere near Centaurus

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皮膚の,皮膚を冒す
狼瘡(ろうそう)(皮膚結核の一種)

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/02/21 00:33:58」(JST)

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Lupus erythematosus is a name given to a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks normal, healthy tissues.^[1] Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs.

Classification

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

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:^[2]^[3]

acute cutaneous lupus erythematosus
subacute cutaneous lupus erythematosus
chronic cutaneous lupus erythematosus
- discoid lupus erythematosus
  - 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)
complement deficiency syndromes
drug-induced lupus erythematosus
neonatal lupus erythematosus
systemic lupus erythematosus

Signs and symptoms

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. Other common symptoms include:

chest pain when taking a deep breath
fatigue
fever with no other cause
general discomfort, uneasiness, or ill feeling (malaise)
hair loss
mouth sores
sensitivity to sunlight
skin rash - a "butterfly" rash in about half people with SLE
swollen lymph nodes^[4]

Photosensitivity

Photosensitivity is a known symptom of lupus, but its relationship to and influence on other aspects of the disease remain to be defined.^[5] Causes of photosensitivity may include:

change in autoantibody location
cytotoxicity
inducing apoptosis with autoantigens in apoptotic blebs
upregulation of adhesion molecules and cytokines
inducing nitric oxide synthase expression
ultraviolet-generated antigenic DNA.
tumor necrosis factor alpha also seems to play a role in the development of photosensitivity.

Genetics

Causes

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, which are largely related to healthy functioning of the immune system. There have been several cases where 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 genes C1, C2, or C4. The influence of sex chromosomes and environmental factors are also noteworthy. Usually, these factors contribute to Lupus by compromising the immune system.^[6]

Age difference

Lupus can develop in any age group with varying results. Typically, the manifestation of the disease tends to be more acute in those affected who are of younger age. Women are more likely to get it than men. It is more common for a Native American, black or Asian person to get Lupus than a Caucasian. Patients with juvenile onset Lupus in particular, are vulnerable to mucocutaneous (alopecia, skin rash, and ulceration of the mucus membranes) manifestations of the disease more so than any other age group. However, patients with late onset Lupus have a much higher mortality rate. Nearly 50% of those with late onset Lupus died of their affliction. This is most likely due to the age of the patients with late onset Lupus since the manifestation of their disease is much less severe than younger patients. Women who are of childbearing age are also particularly at risk.^[7]

Differences in ethnicity

Substantial data have been found to indicate that certain ethnic populations could be more at risk for Lupus Erythematosus, and have a better or worse prognosis. Asian, African, and Native Americans are more likely to get Lupus than Caucasians. Caucasians seem to generally have a more mild 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 ethnicity that had a higher survival rate than Caucasians were 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 problems with malignancies.^[8] "Lupus Facts, Symptoms, Rash, Effects, Complications, and More." WebMD, n.d. Web. 6 October 2014. <http://www.webmd.com/lupus/arthritis-lupus>.

Treatment

Treatment consists primarily of immunosuppressive drugs (e.g., hydroxychloroquine and corticosteroids). 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.^[9]

Epidemiology

Worldwide

an estimated 5 million people worldwide have some form of lupus.^[10]
70% of lupus cases diagnosed are systemic lupus erythematosus.^[10]
20% of people with lupus will have a parent or sibling who already has lupus or may develop lupus.^[10]
about 5% of the children born to individuals with lupus will develop the illness.^[10]

United Kingdom

SLE affects UK females far more than males at a ratio of 7:1. In other words, females are seven times more likely to have the disease.^[11]
The estimated number of UK females with SLE is 21,900, and the number of UK males with lupus is 3000—a total of 24,700, or 0.041% of the population.^[11]
SLE is more common amongst certain ethnic groups than others, especially those of African origin.^[11]

United States

Systemic lupus erythematosus:

occurs from infancy to old age, with peak occurrence between ages 15 and 40.^[9]
affects U.S. females 6 to 10 times more often than males.^[9]
yields limited prevalence data. Estimates vary and range from 1.8 to 7.6 cases per 100,000 persons per year in parts of the continental United States.^[9]

References

^ 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 0-07-144019-4.
^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Disease of the Skin: Clinical Dermatology. (10th ed.). Saunders. Chapter 8. ISBN 0-7216-2921-0.
^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
^ http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001471/
^ Scheinfeld NS, Deleo V. Photosensitivity in lupus erythematosus. Photodermatol Photoimmunol Photomed. 2004;20:272-9. PMID 15379880
^ Kiriakidou, Marianthi."Systemic Lupus Erythematosus." Annals Of Internal Medicine 159.7 (2013): 4-1-4-16. Academic Search Premier. Web. 3 Mar. 2014.
^ "Associations Of Clinical Features And Prognosis With Age At Disease Onset In Patients With Systemic Lupus Erythematosus." Lupus 23.3 (2014): 327-334. Academic Search Premier. Web. 3 Mar. 2014.
^ "Survival In Systemic Lupus Erythematosus, 1995–2010. A Prospective Study In A Danish Community." Lupus 22.11 (2013): 1185-1191. Academic Search Premier. Web. 3 Mar. 2014.
^ ^a ^b ^c ^d http://www.cdc.gov/arthritis/basics/lupus.htm
^ ^a ^b ^c ^d http://www.lupus.org/webmodules/webarticlesnet/templates/new_newsroomreporters.aspx?articleid=247&zoneid=60
^ ^a ^b ^c http://www.arthritisresearchuk.org/arthritis-information/data-and-statistics/lupus.aspx

External links

Lupus Foundation of America
Lupus UK
The Lupus Site

UpToDate Contents

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1. 全身性エリテマトーデスの皮膚粘膜症状 mucocutaneous manifestations of systemic lupus erythematosus
2. 円板状ループスおよび亜急性皮膚ループスの初期マネージメント initial management of discoid lupus and subacute cutaneous lupus
3. 難治性円板状ループスおよび亜急性皮膚ループスのマネージメント management of refractory discoid lupus and subacute cutaneous lupus
4. 薬物誘発性ループス drug induced lupus
5. 成人における全身性エリテマトーデスの診断および鑑別診断 diagnosis and differential diagnosis of systemic lupus erythematosus in adults

English Journal

[Current therapeutic indications of thalidomide and lenalidomide.]

Ordi-Ros J1, Cosiglio FJ2.Author information 1Servicio de Medicina Interna, Hospital Vall d'Hebron, Instituto de Investigación Vall d'Hebron, Universidad Autónoma de Barcelona, Barcelona, España. Electronic address: Jordi@vhebron.net.2Servicio de Clínica Médica, Hospital Provincial del Centenario, Facultad de Ciencias Médicas, Universidad Nacional de Rosario, Rosario, Argentina.AbstractThalidomide is a synthetic glutamic acid derivative first introduced in 1956 in Germany as an over the counter medications. It was thought to be one of the safest sedatives ever produced as it was effective in small doses, was not addictive, and did not have acute side-effects such as motor impairment, but was quickly removed from market after it was linked to cases of severe birth defects. The Food and Drug Administration approved use in the treatment of erythema nodosum leprosum. Further, it was shown its effectiveness in unresponsive dermatological conditions such as actinic prurigo, adult Langerhans cell hystiocytosis, aphthous stomatitis, Behçet syndrome, graft-versus-host disease, cutaneous sarcoidosis, erythema multiforme, Jessner-Kanof lymphocytic infiltration of the skin, Kaposi sarcoma, lichen planus, lupus erythematosus, melanoma, prurigo nodularis, pyoderma gangrenosum and others. In May 2006, it was approved for the treating multiple myeloma. New thalidomide analogues have been developed but lack clinical experience. This paper is a review of the history, pharmacology, mechanism of action, clinical applications and side effects of thalidomide and its analogues.
Medicina clinica.Med Clin (Barc).2014 Apr 22;142(8):360-364. doi: 10.1016/j.medcli.2013.04.038. Epub 2013 Jul 3.
Thalidomide is a synthetic glutamic acid derivative first introduced in 1956 in Germany as an over the counter medications. It was thought to be one of the safest sedatives ever produced as it was effective in small doses, was not addictive, and did not have acute side-effects such as motor impairme
PMID 23830554

Differential management of mild-to-severe psoriasis with biologic drugs: An Italian Delphi consensus expert panel.

Girolomoni G1, Altomare G, Ayala F, Berardesca E, Calzavara Pinton P, Chimenti S, Martini P, Peserico A, Puglisi Guerra A, Antonio Vena G; on behalf on the Delphi working group.Author information 1Department of Medicine, Section of Dermatology and Venereology, University of Verona , Verona , Italy .AbstractAbstract Background: In the management of moderate-to-severe psoriasis, increasingly complex clinical scenarios necessitate practical tools for appropriate biologic therapy selection in individual patients. An Italian Delphi consensus panel provided guidance on biologic use in selected clinical scenarios. Methods: Ten experts defined statements under consideration, which were distributed as an online survey to a dermatologist panel. Plenary discussions of contentious statements were held to achieve consensus. Results: The survey was sent to 30 clinicians. After plenary discussions, consensus was reached on all 20 statements on the following topics: special populations; infections; comorbidities; immunogenicity; extra-cutaneous involvement; pregnancy; and adherence. Three statements required further discussion in order to gain consensus: use of subcutaneous biologics in mild liver impairment (final 94% agreement), use of any biologic in discoid lupus erythematosus (final 100% disagreement), and use of etanercept in patients with history of hypersensitivity reactions to drugs and/or food (final 75% disagreement). Conclusions: This Delphi expert consensus on the use of biologics in psoriasis provides practical recommendations for dermatologists to use when choosing an appropriate biologic in challenging but common clinical scenarios. More data are required to clarify clinical differences of biologic drugs used to treat psoriasis.
The Journal of dermatological treatment.J Dermatolog Treat.2014 Apr 8. [Epub ahead of print]
Abstract Background: In the management of moderate-to-severe psoriasis, increasingly complex clinical scenarios necessitate practical tools for appropriate biologic therapy selection in individual patients. An Italian Delphi consensus panel provided guidance on biologic use in selected clinical scen
PMID 24708158

Cutaneous horn arising from an area of discoid lupus erythematosus on the scalp.

Fatani MI1, Hussain WM, Baltow B, Alsharif S.Author information 1Department of Medicine, Hera General Hospital, Makkah, Saudi Arabia.AbstractA cutaneous horn is a rare clinical condition characterised by a conical projection of hyperkeratotic epidermis. Cutaneous horns most commonly arise from sun-exposed skin in elderly men, but may arise from any part of the body at any age in men and women. When a cutaneous horn forms, it is important to determine the underlying cause. Various skin diseases may present with cutaneous horns including viral warts, actinic keratosis, keratoacanthoma, seborrhoeic keratosis, pyogenic granuloma, discoid lupus erythematosus, verruca vulgaris, Bowen's disease, basal cell carcinoma and squamous cell carcinoma. The underlying pathology is benign in 61.1% of cases, premalignant in 23.2% of cases and malignant in 15.7% of cases. We report a patient with a cutaneous horn arising from an area of discoid lupus erythematosus on the scalp.
BMJ case reports.BMJ Case Rep.2014 Apr 3;2014. pii: bcr2013202322. doi: 10.1136/bcr-2013-202322.
A cutaneous horn is a rare clinical condition characterised by a conical projection of hyperkeratotic epidermis. Cutaneous horns most commonly arise from sun-exposed skin in elderly men, but may arise from any part of the body at any age in men and women. When a cutaneous horn forms, it is important
PMID 24700037