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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/10/01 13:29:04」(JST)

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Pathergy is a skin condition in which a minor trauma such as a bump or bruise leads to the development of skin lesions or ulcers that may be resistant to healing. Pathergy can also lead to ulcerations at the site of surgical incisions. Pathergy was seen with both Behçet's disease and pyoderma gangrenosum.^[1] A highly similar phenomenon known as Koebner occurs in autoimmune diseases such as psoriasis and systemic lupus erythematosus, among others.

Doctors looking toward a diagnosis of Behçet's disease may attempt to induce a pathergy reaction with a test known as a "Skin Prick Test".^[2] The inflammation and ulceration that occurs as a result of pathergy in pyoderma gangrenosum often responds to systemic steroid therapy.

The pathergy reaction is a unique feature of Behçet's disease and, according to the International Study Group for Behcet's Disease, is among the major criteria required for the diagnosis. Different positive pathergy reaction rates in BD have been reported worldwide. When BD-positive groups are studied for pathergy reaction, the pathergy-positive and pathergy-negative BD groups showed a similar male: female ratio, age at disease onset, and mean disease duration. They also exhibited similar HLA-B51 levels and a similar frequency of oral ulcerations in close family members. The mucocutaneous manifestations, systemic disease expression, and severity score were similar in patients with and without the pathergy reaction. The presence of a positive pathergy reaction is not associated with an increased risk for specific mucocutaneous or systemic manifestations of the disease, and probably does not predict a more severe disease course.^[3]

It is also important to note that, while a positive pathergy reaction helps to confirm a specific Behçet's disease diagnosis, a negative reaction does not invalidate a BD diagnosis, because the disease process has to be active at the time of the skin prick test to produce a pathergy reaction. Differences in positive/negative pathergy and severity of the reaction depend on disease activity, ethnicity, type of needle used for the prick test, among other factors.^[2]

References

^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
^ ^a ^b http://www.jimmunol.org/content/177/9/6415.full.pdf
^ Krause I, Molad Y, Mitrani M, Weinberger A (2000). "Pathergy reaction in Behçet's disease: lack of correlation with mucocutaneous manifestations and systemic disease expression". Clin. Exp. Rheumatol. 18 (1): 71–4. PMID 10728447.

UpToDate Contents

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1. ベーチェット症候群の臨床症状と診断 clinical manifestations and diagnosis of behcet syndrome
2. 壊疽性膿皮症：病因、臨床症状、および診断 pyoderma gangrenosum pathogenesis clinical features and diagnosis
3. 壊疽性膿皮症：治療および予後 pyoderma gangrenosum treatment and prognosis
4. 好中球性皮膚症 neutrophilic dermatoses
5. ベーチェット症候群の治療 treatment of behcet syndrome

English Journal

[Recurrent postpartum pyoderma gangrenosum and fatal cardiomyopathy].

Naciri I1, Meziane M2, Benzekri L2, Ghaouti M3, Senouci K2, Hassam B2.
Annales de dermatologie et de venereologie.Ann Dermatol Venereol.2017 Dec 6. pii: S0151-9638(17)31027-X. doi: 10.1016/j.annder.2017.11.006. [Epub ahead of print]
PMID 29223609

The prevalence of Behçet's disease in the north of Jordan: a hospital-based epidemiological survey.

Madanat WY1, Alawneh KM2, Smadi MM3, Saadeh SS2, Omari MM2, Bani Hani AB2, Yazici H4.
Clinical and experimental rheumatology.Clin Exp Rheumatol.2017 Nov-Dec;35 Suppl 108(6):51-54. Epub 2017 Nov 27.
PMID 29224587