eMedicine Specialties > Dermatology > Environmental

Prurigo Nodularis

Author: Daniel J Hogan, MD, Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center
Coauthor(s): Siobahn M Bower, MD, Internal Medicine Resident, Creighton University; Sharron M Mason, MD, Staff Physician, Department of Internal Medicine, University of Kansas School of Medicine; Stephen H Mason, MD, Assistant Professor of Dermatology, Medical College of Georgia
Contributor Information and Disclosures

Updated: Oct 23, 2009

Introduction

Background

In 1909, Hyde and Montgomery1 first described prurigo nodularis as pruritic nodules on the extensor surfaces of the lower extremities in middle-aged women. Prurigo nodularis can be a bothersome-to-debilitating disease, usually seen as multiple, intensely pruritic, excoriated nodules erupting on the extensor surfaces of the limbs secondary to itching or rubbing. In current practice, prurigo nodularis is still a condition of unknown etiology. Many conditions have been reported to induce prurigo nodularis, from internal malignancy to renal failure to psychiatric conditions.

Pathophysiology

Chronic mechanical trauma to the skin causes thickening of the skin proportionate to the trauma. Repetitive rubbing, scratching, and touching (induced by a foreign body or self-induced) results in plaque or nodular lichenification and hyperkeratosis. Pigmentary changes often result from such repetitive trauma to the skin.

With prurigo nodularis, a person feels intense pruritus at discrete points and cannot control the urge to rub or scratch these points on the body. Any abnormality or explanation for the pruritus is unknown; scratching by the individuals who are affected is obvious. The results are discrete, nodular, hyperpigmented/purpuric lesions with surfaces that are scaly, excoriated, and possibly crusted.

Mortality/Morbidity

Prurigo nodularis is benign and does not increase mortality; however, severe morbidity can occur in untreated and even in some treated persons who are affected. Pruritus and the extent of body surface area involved become so great for some patients that they no longer feel functional for work or other everyday activities.

Some conditions associated with prurigo nodularis may cause mortality. Prurigo nodularis has been documented to be much more common in immunocompromised and HIV populations. Some associations have been made of prurigo nodularis with internal malignancy and severely decreased kidney function.

Race

No racial disparity is known for prurigo nodularis.

Sex

Women were formerly believed to have a disproportionate amount of prurigo nodularis compared to men; however, no documented difference exists in frequency between the sexes.

Age

Prurigo nodularis can occur at any age, but it most often occurs in middle-aged and older persons.

Clinical

History

  • Prurigo nodularis patients are most often middle-aged to elderly.
  • Patients with prurigo nodularis invariably complain of a long-standing history of severe, unremitting pruritus.
  • Patients can point out specific sites where they began feeling itchy and where dark-colored nodules formed soon after.
  • Mature nodules rarely increase or decrease in size; spontaneous resolution is even more rare.
  • Prurigo nodularis is usually bilaterally symmetric, with nodules that are either stable or increasing in number.
  • The patient's medical history may be significant for several conditions.
    • Hepatic or renal dysfunction
    • Local trauma or insult to the skin
    • Infection
    • HIV/immunodeficiency2
    • Anxiety or other psychiatric condition
  • Patients may have no significant medical or psychiatric history.
  • The patient's history often reveals a long list of over-the-counter and/or prescribed medications (topical and oral), which usually have produced little or no relief of symptoms.
  • Up to 80% of patients have a personal or family history of atopic dermatitis, asthma, or hay fever (compared with approximately 25% of the normal population).

Physical

  • Prurigo nodularis nodules or papules are 3-20 mm in diameter; they are discrete, scaly, generally symmetric, hyperpigmented or purpuric, and firm.
  • Nodules and papules occur on the extensor surfaces of the arms, the legs, and sometimes the trunk.
  • Prurigo nodularis lesions may show signs of excoriation with flat, umbilicated, or crusted top.
  • Lesions may number from 1-2 to hundreds.
  • The nodule pattern may be follicular.
  • Nodule pattern may be follicular.
  • Upon entering the examination room and while patients' describe the locations of the lesions, patients may scratch or rub the lesions rather than pointing to them.
  • Many prurigo nodularis patients appear very anxious, worried, or even obsessed with the nodules.
Prurigo nodularis. Courtesy of Jeffrey Meffert, M...

Prurigo nodularis. Courtesy of Jeffrey Meffert, MD.

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Prurigo nodularis. Courtesy of Jeffrey Meffert, M...

Prurigo nodularis. Courtesy of Jeffrey Meffert, MD.


Prurigo nodularis. Courtesy of Jeffrey Meffert, M...

Prurigo nodularis. Courtesy of Jeffrey Meffert, MD.

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Prurigo nodularis. Courtesy of Jeffrey Meffert, M...

Prurigo nodularis. Courtesy of Jeffrey Meffert, MD.


Causes

The cause of prurigo nodularis is still unknown. Many associated conditions are known, but their roles as coexisting or preexisting conditions have not been established in causing prurigo nodularis. Notable changes in papules and nodules are increased in certain inflammatory cell types, inflammatory products, and neural hyperplasia.

  • Mast cells and neutrophils are seen in higher-than-normal levels in prurigo nodularis; however, their degranulation products are not increased. Eosinophils are not seen in higher numbers; however, the protein granule products (ie, major basic protein, eosinophil cationic protein, eosinophil-derived neurotoxin) are seen in significantly higher levels.
  • Papillary dermal nerves and Merkel cells are sensory nerves found in the dermis and the epidermis, respectively.3 They are both found in increased numbers in prurigo nodularis. These are neural receptors that sense touch, temperature, pain, and itch. These increases in sensory nerves are not seen in lichen simplex chronicus, another pruritic disease that causes epidermal hyperplasia but in a plaquelike morphology.
  • Calcitonin gene–related peptide and substance P immunoreactive nerves are markedly increased in prurigo nodularis skin compared with normal skin.4 These neuropeptides may mediate the cutaneous neurogenic inflammation and pruritus in prurigo nodularis. In addition, the capsaicin-binding nonselective cation channel known as vanilloid receptor subtype 1 has highly increased expression in epidermal keratinocytes and nerve fibers in prurigo nodularis lesions, but these can be normalized with capsaicin application.
  • Hepatitis C, mycobacteria,5,6,7 Helicobacter pylori, Strongyloides stercoralis,8 and HIV have been reported as infectious etiologies of prurigo nodularis or as associated with prurigo nodularis in case reports or from single-center studies.
  • Interleukin 31, a T-cell–derived cytokine that causes severe pruritus and dermatitis in transgenic mice, is elevated in individuals with prurigo nodularis.9 Interleukin 31 expression in atopic individuals is also rapidly induced by staphylococcal superantigen; however, the link between these findings has not been extensively researched.

More on Prurigo Nodularis

Overview: Prurigo Nodularis
Differential Diagnoses & Workup: Prurigo Nodularis
Treatment & Medication: Prurigo Nodularis
Follow-up: Prurigo Nodularis
Multimedia: Prurigo Nodularis
References
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References

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  38. Stander S, Moormann C, Schumacher M, et al. Expression of vanilloid receptor subtype 1 in cutaneous sensory nerve fibers, mast cells, and epithelial cells of appendage structures. Exp Dermatol. Mar 2004;13(3):129-39. [Medline].

  39. Szeidemann Z, Shanabrough M, Leranth C. Hypothalamic Leu-enkephalin-immunoreactive fibers terminate on calbindin-containing somatospiny cells in the lateral septal area of the rat. J Comp Neurol. Aug 7 1995;358(4):573-83. [Medline].

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Further Reading

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Keywords

prurigo nodularis, Hyde prurigo nodularis, picker’s nodules, lichen simplex chronicus, prurigo nodularis type, atypical nodular form of neurodermatitis circumscripta, lichen corneus obtusus, PN

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Contributor Information and Disclosures

Author

Daniel J Hogan, MD, Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center
Daniel J Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, and Canadian Dermatology Association
Disclosure: Nothing to disclose.

Coauthor(s)

Siobahn M Bower, MD, Internal Medicine Resident, Creighton University
Siobahn M Bower, MD is a member of the following medical societies: American College of Physicians and American Medical Association
Disclosure: Nothing to disclose.

Sharron M Mason, MD, Staff Physician, Department of Internal Medicine, University of Kansas School of Medicine
Disclosure: Nothing to disclose.

Stephen H Mason, MD, Assistant Professor of Dermatology, Medical College of Georgia
Stephen H Mason, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American Society for Dermatologic Surgery, Skin Cancer Foundation, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Medical Editor

Franklin Flowers, MD, Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, University of Florida College of Medicine
Franklin Flowers, MD is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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