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Prurigo nodularis

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Prurigo nodularis
Pruritic papules and scattered nodules can be seen (a) on the legs, (b) on the arms and (c) on the trunk of this patient with prurigo nodularis.
Scattered excoriated nodules across the extensor surface of both hands
SpecialtyDermatology Edit this on Wikidata

Prurigo nodularis (PN), also known as nodular prurigo, is a skin disorder characterized by pruritic (itchy), nodular lesions, which commonly appear on the trunk, arms and legs.[1] Patients often present with multiple excoriated nodules caused by chronic scratching. Although the exact cause of PN is unknown, PN is associated with other dermatologic conditions such as untreated or severe atopic dermatitis and systemic causes of pruritus including liver disease and end stage kidney disease.[2] The goal of treatment in PN is to decrease itching. PN is also known as Hyde prurigo nodularis, or Picker's nodules.[3]

Signs and symptoms

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  • Nodules are discrete, generally symmetric, hyperpigmented and firm. They are greater than 0.5 cm in both width and depth (as opposed to papules which are less than 0.5 cm).
  • The nodules of PN can appear on any part of the body, but generally are found in areas where patients are able to reach to scratch. Patients can exhibit a 'butterfly sign' in which nodules are absent in the mid upper back.[1][4]
  • Nodular lesions are often excoriated from persistent scratching.
  • The nodules in PN are extremely itchy, this sensation can have an impact on patients perceived quality of life[5]
  • Nodule pattern can be follicular.[citation needed]

Causes

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The exact cause of prurigo nodularis is unknown, however, it is thought to be induced by other dermatologic conditions such as severe atopic dermatitis, Becker's nevus,[6] and linear IgA disease.[7] PN is also associated with systemic causes of pruritus such as liver disease,[8] cholestasis, thyroid disease, polycythemia vera, uremia, Hodgkins lymphoma, HIV and kidney failure diseases.[9][10] Psychiatric illnesses have been considered to induce PN, although later research refuted a psychiatric cause for PN. Patients report an ongoing battle to distinguish themselves from those with psychiatric disorders, such as delusions of parasitosis and other psychiatric conditions.[11][12]

Pathophysiology

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Chronic and repetitive scratching, picking, or rubbing of the nodules may result in permanent changes to the skin, including nodular lichenification, hyperkeratosis, hyperpigmentation, and skin thickening. Unhealed, excoriated lesions are often scaly, crusted or scabbed. Many patients report a lack of wound healing even when medications relieve the itching and consequent scratching.[citation needed]

Patients often:[citation needed]

  • seek treatment during middle-age, although PN can occur at any age.
  • have a history of chronic severe pruritus.
  • have a significant medical history for unrelated conditions.
  • develop liver or kidney dysfunctions.
  • develop secondary skin infections.
  • have a personal or family history of atopic dermatitis.
  • have other autoimmune disorders.
  • have low vitamin D levels.

Diagnosis

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Diagnosis is based on visual examination and the presence of itching for greater than 6 weeks duration.

[13] A skin biopsy is often performed to exclude other diseases. Lesion biopsies usually show light inflammation, sometimes with increased numbers of eosinophils.[14] A culture of at least one lesion will rule out staphylococcus infection, which has been significantly linked to atopic dermatitis.[15][16][17]

Treatment

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Prurigo nodularis is hard to treat and therapies include steroids, dupilumab, vitamins, cryosurgery, thalidomide, and UVB light[citation needed]. In the event that staphylococcus or other infection is present, antibiotics have proven effective, but tend to cause more harm than good for this particular disease. A physician may administer a strong dose of prednisone, which will almost immediately stop the itch/scratch cycle. However, cessation of steroids allows relapse to occur, usually within a few weeks. A 2006 study reported improvement in prurigo nodularis with antibiotic therapy.[18]

Azathioprine, an immunosuppressive drug used in organ transplantation and autoimmune diseases belonging to the chemical class of purine analogues, also known by the brand name Imuran, has been reported to be effective.[19][20]

Dupilumab (Dupixent) was approved for medical use in the United States in September 2022.[21] Dupilumab is the first medication approved for the treatment of prurigo nodularis by the US Food and Drug Administration.[21]

Nemolizumab (Nemluvio) was approved for medical use in the United States in August 2024.[22]

History

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Prurigo nodularis was first described by Hyde and Montgomery in 1909.[23]

Notes

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  1. ^ a b Leis M, Fleming P, Lynde CW (May 2021). "Prurigo Nodularis: Review and Emerging Treatments". Skin Therapy Letter. 26 (3): 5–8. ISSN 1201-5989. PMID 34077168.
  2. ^ "Prurigo nodularis: Causes". www.aad.org. Retrieved 13 February 2024.
  3. ^ Frølunde AS, Wiis MA, Ben Abdallah H, Elsgaard S, Danielsen AK, Deleuran M, et al. (13 April 2022). "Non-Atopic Chronic Nodular Prurigo (Prurigo Nodularis Hyde): A Systematic Review of Best-Evidenced Treatment Options". Dermatology. 238 (5): 950–960. doi:10.1159/000523700. ISSN 1018-8665. PMID 35417906.
  4. ^ Huang AH, Williams KA, Kwatra SG (December 2020). "Prurigo nodularis". Journal of the American Academy of Dermatology. 83 (6): 1559–1565. doi:10.1016/j.jaad.2020.04.183. PMID 32454098. S2CID 218910999.
  5. ^ Ingrasci G, El-Kashlan N, Alexis A, Yosipovitch G (1 July 2022). "Chronic itch in African Americans: an unmet need". Archives of Dermatological Research. 314 (5): 405–415. doi:10.1007/s00403-021-02255-6. ISSN 1432-069X. PMID 34129098. S2CID 235429367.
  6. ^ Lockshin BN, Brogan B, Billings S, Billings S (December 2006). "Eczematous dermatitis and prurigo nodularis confined to a Becker's nevus". International Journal of Dermatology. 45 (12): 1465–1466. doi:10.1111/j.1365-4632.2006.02971.x. PMID 17184268. S2CID 32658695.
  7. ^ Torchia D, Caproni M, Del Bianco E, Cozzani E, Ketabchi S, Fabbri P (August 2006). "Linear IgA disease presenting as prurigo nodularis". The British Journal of Dermatology. 155 (2): 479–480. doi:10.1111/j.1365-2133.2006.07315.x. PMID 16882196. S2CID 28166468.
  8. ^ Hazin R, Abu-Rajab Tamimi TI, Abuzetun JY, Zein NN (October 2009). "Recognizing and treating cutaneous signs of liver disease". Cleveland Clinic Journal of Medicine. 76 (10): 599–606. doi:10.3949/ccjm.76A.08113. PMID 19797460.
  9. ^ Hiramanek N (July 2004). "Itch: a symptom of occult disease". Australian Family Physician. 33 (7): 495–499. PMID 15301165.
  10. ^ Berger TG, Hoffman C, Thieberg MD (November 1995). "Prurigo nodularis and photosensitivity in AIDS: treatment with thalidomide". Journal of the American Academy of Dermatology. 33 (5 Pt 1): 837–838. doi:10.1016/0190-9622(95)91846-9. PMID 7593791.
  11. ^ Kieć-Swierczyńska M, Dudek B, Krecisz B, Swierczyńska-Machura D, Dudek W, Garnczarek A, et al. (2006). "[The role of psychological factors and psychiatric disorders in skin diseases]" [The role of psychological factors and psychiatric disorders in skin diseases]. Medycyna Pracy (in Polish). 57 (6): 551–555. PMID 17533993.
  12. ^ Vargas Laguna E, Peña Payero ML, Vargas Márquez A (December 2006). "[Influence of anxiety in diverse cutaneous diseases]" [Influence of anxiety in diverse cutaneous diseases]. Actas Dermo-Sifiliograficas (in Spanish). 97 (10): 637–643. doi:10.1016/s0001-7310(06)73484-6. PMID 17173825.[permanent dead link]
  13. ^ Kwon CD, Khanna R, Williams KA, Kwatra MM, Kwatra SG (26 September 2019). "Diagnostic Workup and Evaluation of Patients with Prurigo Nodularis". Medicines. 6 (4): 97. doi:10.3390/medicines6040097. ISSN 2305-6320. PMC 6963711. PMID 31561504.
  14. ^ Johansson O, Liang Y, Marcusson JA, Reimert CM (August 2000). "Eosinophil cationic protein- and eosinophil-derived neurotoxin/eosinophil protein X-immunoreactive eosinophils in prurigo nodularis". Archives of Dermatological Research. 292 (8): 371–378. doi:10.1007/s004030000142. PMID 10994770. S2CID 9095256.
  15. ^ Gong JQ, Lin L, Lin T, Hao F, Zeng FQ, Bi ZG, et al. (October 2006). "Skin colonization by Staphylococcus aureus in patients with eczema and atopic dermatitis and relevant combined topical therapy: a double-blind multicentre randomized controlled trial". The British Journal of Dermatology. 155 (4): 680–687. doi:10.1111/j.1365-2133.2006.07410.x. PMID 16965415. S2CID 27948096.
  16. ^ Lin YT, Wang CT, Chiang BL (December 2007). "Role of bacterial pathogens in atopic dermatitis". Clinical Reviews in Allergy & Immunology. 33 (3): 167–177. doi:10.1007/s12016-007-0044-5. PMID 18163223. S2CID 25203471.
  17. ^ Guzik TJ, Bzowska M, Kasprowicz A, Czerniawska-Mysik G, Wójcik K, Szmyd D, et al. (April 2005). "Persistent skin colonization with Staphylococcus aureus in atopic dermatitis: relationship to clinical and immunological parameters". Clinical and Experimental Allergy. 35 (4): 448–455. doi:10.1111/j.1365-2222.2005.02210.x. PMID 15836752. S2CID 38030209.
  18. ^ Horiuchi Y, Bae S, Katayama I (April 2006). "Uncontrollable prurigo nodularis effectively treated by roxithromycin and tranilast". Journal of Drugs in Dermatology. 5 (4): 363–365. PMID 16673805.
  19. ^ Briatico G, Scharf C, Di Brizzi EV, Pellerone S, Buononato D, Squadrilli F, et al. (2023). "Amitriptyline and azathioprine: an effective therapeutic approach in prurigo nodularis resistant to conventional therapies". JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 21 (3): 291–293. doi:10.1111/ddg.14975. ISSN 1610-0379. PMID 36730609.
  20. ^ Lear J, English J, Smith A (1996). "Nodular prurigo responsive to azathioprine (letter)". British Journal of Dermatology. 134 (6): 1151. doi:10.1111/j.1365-2133.1996.tb07964.x. PMID 8763446.
  21. ^ a b "FDA approves first treatment for prurigo nodularis". U.S. Food and Drug Administration (FDA). 29 September 2022.
  22. ^ "Galderma Receives U.S. FDA Approval for Nemluvio (nemolizumab) for Adult Patients Living With Prurigo Nodularis" (Press release). Galderma. 13 August 2024. Retrieved 14 August 2024 – via Business Wire.
  23. ^ Hyde JN, Montgomery FH: A practical treatise on disease of the skin for the use of students and practitioners. 1909; 174–175.
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