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Familial pityriasis rubra pilaris

MedGen UID:
443914
Concept ID:
C2930842
Disease or Syndrome
Synonym: Pityriasis rubra pilaris--familial type
 
Monarch Initiative: MONDO:0008251

Definition

A rare chronic papulosquamous disorder of unknown etiology characterized by small follicular papules, scaly red-orange patches, and palmoplantar hyperkeratosis, which may progress to plaques or erythroderma. Although most of the cases are sporadic and acquired, a familial form of the disease exists. [from MONDO]

Recent clinical studies

Etiology

Hill CR, Theos A
Dermatol Clin 2019 Apr;37(2):229-239. doi: 10.1016/j.det.2018.11.004. PMID: 30850045
Lwin SM, Hsu CK, Liu L, Huang HY, Levell NJ, McGrath JA
Br J Dermatol 2018 Apr;178(4):969-972. Epub 2017 Oct 1 doi: 10.1111/bjd.15462. PMID: 28301045

Diagnosis

Hill CR, Theos A
Dermatol Clin 2019 Apr;37(2):229-239. doi: 10.1016/j.det.2018.11.004. PMID: 30850045
Craiglow BG, Boyden LM, Hu R, Virtanen M, Su J, Rodriguez G, McCarthy C, Luna P, Larralde M, Humphrey S, Holland KE, Hogeling M, Hidalgo-Matlock B, Ferrari B, Fernandez-Faith E, Drolet B, Cordoro KM, Bowcock AM, Antaya RJ, Ashack K, Ashack RJ, Lifton RP, Milstone LM, Paller AS, Choate KA
J Am Acad Dermatol 2018 Sep;79(3):487-494. Epub 2018 Mar 1 doi: 10.1016/j.jaad.2018.02.034. PMID: 29477734Free PMC Article
Inoue N, Dainichi T, Fujisawa A, Nakano H, Sawamura D, Kabashima K
J Dermatol 2016 Feb;43(2):187-9. Epub 2015 Jul 1 doi: 10.1111/1346-8138.13008. PMID: 26130407
Mercer JM, Pushpanthan C, Anandakrishnan C, Landells ID
J Cutan Med Surg 2013 Jul-Aug;17(4):226-32. doi: 10.2310/7750.2012.12018. PMID: 23815954
Vanderhooft SL, Francis JS, Holbrook KA, Dale BA, Fleckman P
Arch Dermatol 1995 Apr;131(4):448-53. PMID: 7726588

Therapy

Chen MKY, Flanagan AL, Sebaratnam DF, Gu Y
Australas J Dermatol 2024 Mar;65(2):185-214. Epub 2023 Dec 21 doi: 10.1111/ajd.14197. PMID: 38126177
Hill CR, Theos A
Dermatol Clin 2019 Apr;37(2):229-239. doi: 10.1016/j.det.2018.11.004. PMID: 30850045
Craiglow BG, Boyden LM, Hu R, Virtanen M, Su J, Rodriguez G, McCarthy C, Luna P, Larralde M, Humphrey S, Holland KE, Hogeling M, Hidalgo-Matlock B, Ferrari B, Fernandez-Faith E, Drolet B, Cordoro KM, Bowcock AM, Antaya RJ, Ashack K, Ashack RJ, Lifton RP, Milstone LM, Paller AS, Choate KA
J Am Acad Dermatol 2018 Sep;79(3):487-494. Epub 2018 Mar 1 doi: 10.1016/j.jaad.2018.02.034. PMID: 29477734Free PMC Article
Lwin SM, Hsu CK, Liu L, Huang HY, Levell NJ, McGrath JA
Br J Dermatol 2018 Apr;178(4):969-972. Epub 2017 Oct 1 doi: 10.1111/bjd.15462. PMID: 28301045
Vasher M, Smithberger E, Lien MH, Fenske NA
J Drugs Dermatol 2010 Jul;9(7):844-50. PMID: 20677542

Prognosis

Inoue N, Dainichi T, Fujisawa A, Nakano H, Sawamura D, Kabashima K
J Dermatol 2016 Feb;43(2):187-9. Epub 2015 Jul 1 doi: 10.1111/1346-8138.13008. PMID: 26130407
Mercer JM, Pushpanthan C, Anandakrishnan C, Landells ID
J Cutan Med Surg 2013 Jul-Aug;17(4):226-32. doi: 10.2310/7750.2012.12018. PMID: 23815954

Clinical prediction guides

Chen MKY, Flanagan AL, Sebaratnam DF, Gu Y
Australas J Dermatol 2024 Mar;65(2):185-214. Epub 2023 Dec 21 doi: 10.1111/ajd.14197. PMID: 38126177
Inoue N, Dainichi T, Fujisawa A, Nakano H, Sawamura D, Kabashima K
J Dermatol 2016 Feb;43(2):187-9. Epub 2015 Jul 1 doi: 10.1111/1346-8138.13008. PMID: 26130407
Vanderhooft SL, Francis JS, Holbrook KA, Dale BA, Fleckman P
Arch Dermatol 1995 Apr;131(4):448-53. PMID: 7726588

Recent systematic reviews

Chen MKY, Flanagan AL, Sebaratnam DF, Gu Y
Australas J Dermatol 2024 Mar;65(2):185-214. Epub 2023 Dec 21 doi: 10.1111/ajd.14197. PMID: 38126177

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