Prurigo nodularis

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Classification according to ICD-10
L28.1 Prurigo nodularis
ICD-10 online (WHO version 2019)

The prurigo nodular is a rare skin disease with itching ( prurigo ) skin nodules usually on the limbs . The disease is a sub-form of chronic prurigo and is viewed as the maximum form of prurigo simplex subacuta . Of all forms of itching, it is considered the most severe.

Synonyms are: Latin Lichen obtusus corneus; Urticaria perstans chronica papulosa; Urticaria perstans verrucosa; Lichenificatio maculopapulosa; Lichenificatio nodularis; Tuberosis cutis pruriginosa; Lichen corneus disseminatus; Neurodermatitis nodosa; Eccema verrucosum callosum; Papulae obtusae; Eccema verrucosum callosum nodulare ; nodular prurigo; English Picker’s nodule; Hyde prurigo nodularis

The first description was in 1909 by James Nevins Hyde (1840-1910) and FH Montgomery .

distribution

The frequency is given as less than 1 in 1,000,000. Both sexes are affected, older people somewhat more frequently. Darker skin and African descent are associated with a higher risk. In the USA, a disproportionately large number of African-Americans are affected, while the nodules in "whites" tend to reach deeper - and possibly express a molecular heterogeneity .

root cause

The cause is still unclear. Prurigo nodularis can be associated with a number of chronic diseases, such as diabetes mellitus , HIV or chronic kidney disease . It is also more common in atopic dermatitis , lymphoma of the skin, infection of the skin with mycobacteria, or bullous skin diseases.

In the pathogenesis of prurigo nodularis also a form of "neuronal is sensitization " in front, whereby the itch-process forming nerve cells are triggered and activated, and thereby a "itch-scratch cycle" ( itch-scratch-cyclus ) entertained. An inflammatory reaction in the skin and neural plasticity also play a role; histopathologically, changes in the nerve fibers in the skin and inflammatory cells in the dermis can be found . The itching in this disease can be triggered by the release of tryptase , interleukin 31 , prostaglandins , the eosinophil cationic protein and neuropeptides by inflammatory cells , mast cells and nerve fibers.

In particular, an up to 50-fold increase in the production of interleukin-31 mRNA was observed in the nodules. Interleukin 31 is mainly released by activated CD4- positive T cells , which have a pro-inflammatory effect, and has been identified as a trigger for pruritus in atopic dermatitis and prurigo nodularis.

Interleukin 31 has already been referred to as the "pruritus cytokine" and binds to a heterodimeric receptor consisting of the interleukin 31 receptor A and the oncostatin M receptor β subunit, OSMRβ. The IL-31 receptor A is most strongly expressed in the sensory nerve cells of the spinal ganglia of the spinal cord , which also have increased co-expression of pruritus sensors and inflammatory mediators of neurogenic inflammation, which include TRPV-1 and NPPB . Interleukin 31 also induces phosphorylation and thus activation of the signal transducers of the JAK-STAT signaling pathway JAK1 and JAK2, which are known as mediators of pruritus .

Clinical manifestations

The very itchy nodules are hyperkeratotic (coarse), covered with scabs or scratched, and light red to pale red (with light pigmentation type). They are a few millimeters in size, but can also reach 2-3 cm. Only a few nodules rarely occur, up to several hundred nodules can occur. The nodules are seldom found only in a circumscribed region; they are usually generalized. Then there is often a symmetrical appearance, especially on the extensor sides of the arms and legs, as well as on the trunk. Usually the face, soles of the feet and palms are not affected.

Secondary infections can also result from scratching .

Differential diagnosis

Must be distinguished are other Prurigo -forms as prurigo chronica multiformis , Prurigo subacuta, atopic eczema , lichen planus verrucosus and Prurigoform of bullous pemphigoid .

therapy

There is no causal therapy and treatment is only symptomatic to reduce itching and to prevent and treat consequences such as wounds and secondary infections.

In almost all patients, topical cortisone preparations fail after initial slight successes, and antihistamines are also ineffective. Most patients also experience no relief from attempts at methotrexate , gabapentin , pregabalin, or phototherapy . The only systemic drugs that show any significant effect are cyclosporine and thalidomide , but with significant undesirable effects.

Especially with prurigo nodularis, patients suffer more from anxiety , depression and have more thoughts of suicide .

A new approach is the treatment with nemolizumab, which in 2020 showed good results in a phase II study of the University Clinic Münster in the context of drug approval , but has not yet been approved. This humanized antibody against the human interleukin-31 receptor was injected subcutaneously on a weekly basis and compared with placebo in a double-blind randomized controlled trial . The itch score decreased by a significant 53% after four weeks compared to 20% in the placebo group, while the difference after twelve weeks was probably not significant due to the small population (61% compared to 27%). However, the effect set in quickly and was long-lasting (after 18 weeks, six weeks after the last injection: still 58% against 26%), the number of nodules also decreased, more healed nodules appeared, and sleep and quality of life were significantly improved. Abdominal pain and diarrhea as well as musculoskeletal pain increased with nemolizumab.

Web links

Prurigo nodularis. Competence Center for Chronic Pruritus (KCP) at the University Hospital Münster (UKM)

literature

  • T. Böhme, T. Heitkemper, T. Mettang, NQ Phan, S. Ständer: Clinical characteristics and prurigo nodularis in nephrogenic pruritus. In: The dermatologist ; Journal of Dermatology, Venereology, and Allied Areas , Volume 65, No. 8, August 2014, pp. 714-720, doi: 10.1007 / s00105-014-2756-9 , PMID 25113331 .
  • C. Zeidler, S. Ständer: Therapy of prurigo nodularis. In: The dermatologist; Journal of Dermatology, Venereology, and Related Fields , Volume 65, No. 8, August 2014, pp. 709-713, doi: 10.1007 / s00105-014-2757-8 , PMID 25113330 .
  • G. Schneider, J. Hockmann, A. Stumpf: Psychosomatic aspects of prurigo nodularis. In: The dermatologist; Journal of Dermatology, Venereology, and Related Fields , Volume 65, No. 8, August 2014, pp. 704-708, doi: 10.1007 / s00105-014-2758-7 , PMID 25113329 .
  • U. Raap, C. Günther: Pathogenesis of Prurigo nodularis. In: The dermatologist; Journal of Dermatology, Venereology, and Allied Fields , Volume 65, No. 8, August 2014, pp. 691-696, doi: 10.1007 / s00105-014-2754-y , PMID 25113327 .

Individual evidence

  1. ↑ Peter Altmeyer u. a .: prurigo nodularis L28.1. In: Altmeyers Encyclopedia (Dermatology). January 10, 2020, accessed March 7, 2020 .
  2. ↑ a b c d Sonja Ständer, Gil Yosipovitch, Franz J. Legat, Jean-Philippe Lacour, Carle Paul, Joanna Narbutt, Thomas Bieber, Laurent Misery, Andreas Wollenberg, Adam Reich, Faiz Ahmad, Christophe Piketty: Trial of Nemolizumab in Moderate -to-Severe Prurigo Nodularis , New England Journal of Medicine 2020, Volume 382, ​​Issue 8, February 20, 2020, pages 706-716, DOI: 10.1056 / NEJMoa1908316
  3. ↑ JN Hyde, FH Montgomery: A practical treatise on disease of the skin for the use of students and practitioners. Lea & Febiger, Philadelphia, 1909, pp. 174-175.
  4. ↑ a b c Shawn G. Kwatra: Breaking the Itch-Scratch Cycle in Prurigo Nodularis , New England Journal of Medicine 2020, Volume 382, ​​Issue 8 of February 20, 2020, pages 757-758, DOI: 10.1056 / NEJMe1916733
  5. ↑ Amarateedha Prak LeCourt: Prurigo Nodularis. In: Medscape. June 4, 2019, accessed March 7, 2020 .
  6. ↑ T. Mettang, A. Vonend, U. Raap: Prurigo nodularis in dermatoses and systemic diseases. In: The dermatologist ; Journal of Dermatology, Venereology, and Allied Areas , Volume 65, No. 8, August 2014, pp. 697-703, doi: 10.1007 / s00105-014-2755-x , PMID 25113328 .