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Prurigo nodularis

MedGen UID:
120471
Concept ID:
C0263353
Disease or Syndrome
Synonyms: Hyde's disease; Nodular prurigo; Picker's nodules; prurigo nodularis
SNOMED CT: Prurigo nodularis (63501000); Hyde's disease (63501000); Nodular prurigo (63501000); Picker's nodules (63501000)
 
Monarch Initiative: MONDO:0026045

Definition

Prurigonodularis (PN) is a skin disease in which hard crusty lumps are formed on the skin that itches intensely. Repetitive rubbing, scratching, and touching results in more lesions in the skin. The itching is so intense that people scratch themselves to the point of bleeding or pain. The lumps formed in the skin are hard, and have about a half inch across, with a dry and rough top that is often scratched open. They tend to be located in the areas most easily reached and are worse on the outer sides: arms, shoulders and legs. The trunk, face and even palms can also be affected. The exact cause is unknown but some factors triggering PN include liver and kidneys problems, nervous and mental conditions and other skin diseases. Prurigo nodulares, in some cases, can be seen in other diseases such as lymphoma, chronic autoimmune cholestatic hepatitis, HIV infection, severe anemia,or a chronic kidney disease-related itching known as uremic pruritus. Treatment is very difficult, and may include corticoids, antihistaminic and other medication such as thalidomide and similar (Lenalidomide). In some cases, cryotherapy, photochemotherapy and habit reversal therapy for the itch-scratch cycle has improved the symptoms. PN can last for many years, and the itching is so intense that may affect all the everyday activities. [from MONDO]

Professional guidelines

PubMed

Williams KA, Roh YS, Brown I, Sutaria N, Bakhshi P, Choi J, Gabriel S, Chavda R, Kwatra SG
Expert Rev Clin Pharmacol 2021 Jan;14(1):67-77. Epub 2020 Dec 11 doi: 10.1080/17512433.2021.1852080. PMID: 33191806
Elmariah S, Kim B, Berger T, Chisolm S, Kwatra SG, Mollanazar N, Yosipovitch G
J Am Acad Dermatol 2021 Mar;84(3):747-760. Epub 2020 Jul 15 doi: 10.1016/j.jaad.2020.07.025. PMID: 32682025
Williams KA, Huang AH, Belzberg M, Kwatra SG
J Am Acad Dermatol 2020 Dec;83(6):1567-1575. Epub 2020 May 24 doi: 10.1016/j.jaad.2020.04.182. PMID: 32461078

Recent clinical studies

Etiology

Patel JR, Joel MZ, Lee KK, Kambala A, Cornman H, Oladipo O, Taylor M, Imo BU, Ma EZ, Manjunath J, Kollhoff AL, Deng J, Parthasarathy V, Cravero K, Marani M, Szeto M, Zhao R, Sankararaman S, Li R, Henry S, Pritchard T, Rebecca V, Kwatra MM, Ho WJ, Dong X, Kang S, Kwatra SG
J Invest Dermatol 2024 Jul;144(7):1568-1578.e5. Epub 2024 Jan 20 doi: 10.1016/j.jid.2023.12.021. PMID: 38246584
Kwatra SG, Yosipovitch G, Legat FJ, Reich A, Paul C, Simon D, Naldi L, Lynde C, De Bruin-Weller MS, Nahm WK, Sauder M, Gharib R, Barbarot S, Szepietowski JC, Conrad C, Fleischer A, Laquer VT, Misery L, Serra-Baldrich E, Lapeere H, Ahmad F, Jabbar Lopez ZK, Piketty C, Ständer S; OLYMPIA 2 Investigators
N Engl J Med 2023 Oct 26;389(17):1579-1589. doi: 10.1056/NEJMoa2301333. PMID: 37888917
Napolitano M, Fabbrocini G, Martora F, Genco L, Noto M, Patruno C
Dermatol Ther 2022 Dec;35(12):e15901. Epub 2022 Oct 13 doi: 10.1111/dth.15901. PMID: 36200594Free PMC Article
Ständer S, Yosipovitch G, Legat FJ, Lacour JP, Paul C, Narbutt J, Bieber T, Misery L, Wollenberg A, Reich A, Ahmad F, Piketty C
N Engl J Med 2020 Feb 20;382(8):706-716. doi: 10.1056/NEJMoa1908316. PMID: 32074418
Peckruhn M, Elsner P, Tittelbach J
J Dtsch Dermatol Ges 2019 Oct;17(10):1039-1051. Epub 2019 Sep 27 doi: 10.1111/ddg.13943. PMID: 31562692

Diagnosis

Müller S, Zeidler C, Ständer S
Am J Clin Dermatol 2024 Jan;25(1):15-33. Epub 2023 Sep 17 doi: 10.1007/s40257-023-00818-z. PMID: 37717255Free PMC Article
Napolitano M, Fabbrocini G, Martora F, Genco L, Noto M, Patruno C
Dermatol Ther 2022 Dec;35(12):e15901. Epub 2022 Oct 13 doi: 10.1111/dth.15901. PMID: 36200594Free PMC Article
Elmariah S, Kim B, Berger T, Chisolm S, Kwatra SG, Mollanazar N, Yosipovitch G
J Am Acad Dermatol 2021 Mar;84(3):747-760. Epub 2020 Jul 15 doi: 10.1016/j.jaad.2020.07.025. PMID: 32682025
Williams KA, Huang AH, Belzberg M, Kwatra SG
J Am Acad Dermatol 2020 Dec;83(6):1567-1575. Epub 2020 May 24 doi: 10.1016/j.jaad.2020.04.182. PMID: 32461078
Huang AH, Williams KA, Kwatra SG
J Am Acad Dermatol 2020 Dec;83(6):1559-1565. Epub 2020 May 23 doi: 10.1016/j.jaad.2020.04.183. PMID: 32454098

Therapy

Müller S, Zeidler C, Ständer S
Am J Clin Dermatol 2024 Jan;25(1):15-33. Epub 2023 Sep 17 doi: 10.1007/s40257-023-00818-z. PMID: 37717255Free PMC Article
Olbrich H, Sadik CD, Ludwig RJ, Thaçi D, Boch K
Biomolecules 2023 Mar 31;13(4) doi: 10.3390/biom13040634. PMID: 37189381Free PMC Article
Muñoz-Bellido FJ, Moreno E, Dávila I
J Investig Allergol Clin Immunol 2022 Apr 19;32(2):97-115. Epub 2021 Mar 3 doi: 10.18176/jiaci.0682. PMID: 33661102
Leis M, Fleming P, Lynde CW
Skin Therapy Lett 2021 May;26(3):5-8. PMID: 34077168
Williams KA, Huang AH, Belzberg M, Kwatra SG
J Am Acad Dermatol 2020 Dec;83(6):1567-1575. Epub 2020 May 24 doi: 10.1016/j.jaad.2020.04.182. PMID: 32461078

Prognosis

Vasavda C, Wan G, Szeto MD, Marani M, Sutaria N, Rajeh A, Lu C, Lee KK, Nguyen NTT, Adawi W, Deng J, Parthasarathy V, Bordeaux ZA, Taylor MT, Alphonse MP, Kwatra MM, Kang S, Semenov YR, Gusev A, Kwatra SG
J Invest Dermatol 2023 Dec;143(12):2416-2426.e1. Epub 2023 May 26 doi: 10.1016/j.jid.2023.04.033. PMID: 37245863Free PMC Article
Zeidler C, Raap U, Witte F, Ständer S
J Allergy Clin Immunol 2023 Jul;152(1):1-10. Epub 2023 May 11 doi: 10.1016/j.jaci.2023.04.018. PMID: 37178730
Napolitano M, Fabbrocini G, Martora F, Genco L, Noto M, Patruno C
Dermatol Ther 2022 Dec;35(12):e15901. Epub 2022 Oct 13 doi: 10.1111/dth.15901. PMID: 36200594Free PMC Article
Ryczek A, Reich A
Acta Derm Venereol 2020 May 28;100(10):adv00155. doi: 10.2340/00015555-3518. PMID: 32421199Free PMC Article
Yew YW, Thyssen JP, Silverberg JI
J Am Acad Dermatol 2019 Feb;80(2):390-401. Epub 2018 Oct 1 doi: 10.1016/j.jaad.2018.09.035. PMID: 30287309

Clinical prediction guides

Müller S, Zeidler C, Ständer S
Am J Clin Dermatol 2024 Jan;25(1):15-33. Epub 2023 Sep 17 doi: 10.1007/s40257-023-00818-z. PMID: 37717255Free PMC Article
Kwatra SG, Yosipovitch G, Legat FJ, Reich A, Paul C, Simon D, Naldi L, Lynde C, De Bruin-Weller MS, Nahm WK, Sauder M, Gharib R, Barbarot S, Szepietowski JC, Conrad C, Fleischer A, Laquer VT, Misery L, Serra-Baldrich E, Lapeere H, Ahmad F, Jabbar Lopez ZK, Piketty C, Ständer S; OLYMPIA 2 Investigators
N Engl J Med 2023 Oct 26;389(17):1579-1589. doi: 10.1056/NEJMoa2301333. PMID: 37888917
Wang L, Wang B, Kou E, Du L, Zhu Y
Front Immunol 2023;14:1259515. Epub 2023 Sep 22 doi: 10.3389/fimmu.2023.1259515. PMID: 37809065Free PMC Article
Arrieta A, Jaka A, Del Alcázar E, Blanco M, Carrascosa JM
Actas Dermosifiliogr (Engl Ed) 2021 Apr;112(4):339-344. Epub 2020 Nov 19 doi: 10.1016/j.ad.2020.11.007. PMID: 33221272
Ständer S, Yosipovitch G, Legat FJ, Lacour JP, Paul C, Narbutt J, Bieber T, Misery L, Wollenberg A, Reich A, Ahmad F, Piketty C
N Engl J Med 2020 Feb 20;382(8):706-716. doi: 10.1056/NEJMoa1908316. PMID: 32074418

Recent systematic reviews

McCampbell LE, Zaino ML, Ranpariya M, Patel T, Feldman SR
J Cutan Med Surg 2023 Nov;27(6):641-645. Epub 2023 Nov 21 doi: 10.1177/12034754231211797. PMID: 37987710
Ferreira BR, Misery L
Acta Derm Venereol 2023 Aug 22;103:adv8488. doi: 10.2340/actadv.v103.8488. PMID: 37606153Free PMC Article
Olbrich H, Sadik CD, Ludwig RJ, Thaçi D, Boch K
Biomolecules 2023 Mar 31;13(4) doi: 10.3390/biom13040634. PMID: 37189381Free PMC Article
Frølunde AS, Wiis MAK, Ben Abdallah H, Elsgaard S, Danielsen AK, Deleuran M, Vestergaard C
Dermatology 2022;238(5):950-960. Epub 2022 Apr 13 doi: 10.1159/000523700. PMID: 35417906
Yew YW, Thyssen JP, Silverberg JI
J Am Acad Dermatol 2019 Feb;80(2):390-401. Epub 2018 Oct 1 doi: 10.1016/j.jaad.2018.09.035. PMID: 30287309

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