KEGG   DISEASE: 毛孔性紅色粃糠疹

Pityriasis rubra pilaris (PRP) is a spectrum of rare chronic inflammatory disorders with papulosquamous eruptions of unknown cause. The prototypical clinical characteristics of PRP are follicular hyperkeratotic papules on an erythematous base that can eventually coalesce to large red plaques. Its classification into five subgroups is based on age at onset, clinical course, morphologic features, and prognosis. More than 50% of patients are best classified as type I with adult-onset PRP. It is comprised of widespread, follicular papules and plaques and tends to clear spontaneously in 80% of patients in one to three years. Type II, which is the atypical adult subtype, tends to be more chronic and presents with areas of alopecia, eczematous patches, and a palmoplantar keratoderma. The classic juvenile type III is observed in only 10% of patients. It is similar clinically to type I but occurs in children and tends to remit in one year. Type IV, which is the circumscribed, juvenile subtype, presents with sharply-demarcated areas of follicular hyperkeratosis and erythema of the elbows and knees. An estimated 5% of children with PRP develop an atypical form classified as juvenile type V. Most familial cases that are published belong to this group. Recently, the designation of a new category of PRP (type VI) has been proposed that is characterized by the presence of HIV infection with different clinical features and a poorer prognosis. Treatment of PRP can be difficult, and no standardized approach has been established, although systemic retinoids are considered first-line therapy. Cyclosporin, methotrexate, and azathioprine are alternatives.
ヒト疾患 [BR:jp08402]
   H01659  毛孔性紅色粃糠疹
ICD-11 による疾患分類 [BR:jp08403]
 14 Diseases of the skin
  Inflammatory dermatoses
   Papulosquamous dermatoses
    EA94  Pityriasis rubra pilaris
     H01659  毛孔性紅色粃糠疹
CARD14 [HSA:79092] [KO:K20913]
ベタメタゾンリン酸エステルナトリウム [DR:D00972]
トリアムシノロンアセトニド [DR:D00983]
トリアムシノロン [DR:D00385]
ベタメタゾン [DR:D00244]
プレドニゾロン [DR:D00472]
プレドニゾロンコハク酸エステルナトリウム [DR:D01239]
デキサメタゾンプロピオン酸エステル [DR:D01632]
ベタメタゾン吉草酸エステル [DR:D01357]
ベタメタゾンジプロピオン酸エステル [DR:D01637]
ジフロラゾン酢酸エステル [DR:D01327]
タカルシトール水和物 [DR:D01472]
エトレチナート [DR:D00316]
ICD-11: EA94
ICD-10: L44.0
MeSH: D010916
OMIM: 173200
Leger M, Newlove T, Robinson M, Patel R, Meehan S, Ramachandran S
Pityriasis rubra pilaris.
Dermatol Online J 18:14 (2012)
Ivanova K, Itin P, Haeusermann P
Pityriasis rubra pilaris: treatment with biologics - a new promising therapy?
Dermatology 224:120-5 (2012)
Klein A, Landthaler M, Karrer S
Pityriasis rubra pilaris: a review of diagnosis and treatment.
Am J Clin Dermatol 11:157-70 (2010)
Fuchs-Telem D, Sarig O, van Steensel MA, Isakov O, Israeli S, Nousbeck J, Richard K, Winnepenninckx V, Vernooij M, Shomron N, Uitto J, Fleckman P, Richard G, Sprecher E
Familial pityriasis rubra pilaris is caused by mutations in CARD14.
Am J Hum Genet 91:163-70 (2012)

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