Skin Picking in People with Prader–Willi Syndrome: Phenomenology and Management

Abstract

Skin picking is highly prevalent in people with Prader–Willi syndrome (PWS). This study addressed the temporal (frequency, duration) and wider characteristics (e.g. type of skin picked, apparent motivations, or management strategies) of skin picking to inform intervention strategies. Nineteen parents/carers who observe skin picking shown by the person they care for completed a semi-structured interview. Results were consistent with previous research but advanced the field by finding that most participants picked skin with an imperfection and that parents/carers most commonly use distraction as a management strategy. Interventions that are behavioural, support emotion regulation and/ or are used in the typically developing population are therefore likely to be beneficial for future research.

This is a preview of subscription content, log in to check access.

References

  1. Akefeldt, A., & Gillberg, C. (1999). Behavior and personality characteristics of children and young adults with Prader–Willi syndrome: A controlled study. Journal of the American Academy of Child & Adolescent Psychiatry,38(6), 761–769.

    Article  Google Scholar 

  2. American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (5th ed.). Washington, DC: American Psychiatric Association.

    Google Scholar 

  3. Banga, A., & Connor, D. F. (2012). Effectiveness of naltrexone for treating pathologic skin picking behavior in an adolescent with Prader–Willi syndrome. Journal of Child and Adolescent Psychopharmacology,22(5), 396–398.

    PubMed  Article  Google Scholar 

  4. Beck, A. T. (1967). Depression causes and treatment. Philadelphia, PA: University of Pennsylvania Press.

    Google Scholar 

  5. Benjamin, E., & Buot-Smith, T. (1993). Naltrexone and fluoxetine in Prader–Willi syndrome. Journal of the American Academy of Child & Adolescent Psychiatry,32(4), 870–873.

    Article  Google Scholar 

  6. Boer, H., & Clarke, D. (1999). Development and behaviour in genetic syndromes: Prader-Willi syndrome. Journal of Applied Research in Intellectual Disabilities, 12, 294–301.

    Article  Google Scholar 

  7. Bull, L. E. (2014). Understanding and changing behaviour in Prader-Willi syndrome. Unpublished PhD thesis. University of Birmingham, UK.

  8. Bull, L. E. (2015). Self-injurious behaviour in people with Prader-Willi syndrome. Unpublished thesis. University of Birmingham, UK.

  9. Bull, L. E., Oliver, C., Tunnicliffe, P. L., & Woodcock, K. A. (2015). An informant report behavior diary for measuring temper outbursts in an intervention setting. Journal of Developmental and Physical Disabilities,27, 1–16.

    Article  Google Scholar 

  10. Buono, S., Palmigiano, M., Scannella, F., Occhipinti, P., & Greco, D. (2005). Self-injury and Präder–Willi syndrome. Journal of Policy and Practice in Intellectual Disabilities,2, 256–259.

    Article  Google Scholar 

  11. Buono, S., Scannella, F., & Palmigiano, M. B. (2010). Self-injurious behavior: A comparison between Prader–Willi syndrome, down syndrome and autism. Life Span and Disability,13(2), 187–201.

    Google Scholar 

  12. Cassidy, S. B., & Driscoll, D. J. (2009). Prader–Willi syndrome. European Journal of Human Genetics,17, 3–13.

    PubMed  Article  Google Scholar 

  13. Cassidy, S. B., Forsythe, M., Heeger, S., Nicholls, R. D., Schork, N., Benn, P., et al. (1997). Comparison of phenotype between patients with Prader–Willi syndrome due to deletion 15q and uniparental disomy 15. American Journal of Medical Genetics,68, 433–440.

    PubMed  Article  Google Scholar 

  14. Didden, R., Korzilius, H., & Curfs, L. M. G. (2007). Skin-picking in individuals with Prader–Willi syndrome: Prevalence, functional assessment, and its comorbidity with compulsive and self-injurious behaviours. Journal of Applied Research in Intellectual Disabilities,20, 409–419.

    Article  Google Scholar 

  15. Didden, R., Proot, I., Lancioni, G. E., van Os, R., & Curfs, L. M. G. (2008). Individuals with Prader–Willi syndrome and their perceptions of skin-picking behaviour. British Journal of Developmental Disabilities,54, 123–130.

    Article  Google Scholar 

  16. Dykens, E. M., Cassidy, S. B., & King, B. H. (1999). Maladaptive behavior differences in Prader–Willi syndrome due to paternal deletion versus maternal uniparental disomy. American Journal on Mental Retardation,104, 67–77.

    PubMed  Article  Google Scholar 

  17. Dykens, E. M., Hodapp, R. M., Walsh, K., & Nash, L. J. (1992). Adaptive and maladaptive behavior in Prader–Willi syndrome. Journal of the American Academy of Child & Adolescent Psychiatry,31(6), 1131–1136.

    Article  Google Scholar 

  18. Eccleston, C., & Crombez, G. (1999). Pain demands attention: A cognitive–affective model of the interruptive function of pain. Psychological Bulletin,125(3), 356.

    PubMed  Article  Google Scholar 

  19. Feurer, I. D., Dimitropoulos, A., Stone, W. L., Roof, E., Butler, M. G. & Thompson, T. (1998). The latent variable structure of the Compulsive Behaviour Checklist in people with Prader-Willi syndrome. Journal on Intellectual Disability Research,42(6), 472–480.

    Article  Google Scholar 

  20. Gelinas, B. L., & Gagnon, M. M. (2013). Pharmacological and psychological treatments of pathological skin-picking: A preliminary meta-analysis. Journal of Obsessive-Compulsive and Related Disorders,2(2), 167–175.

    Article  Google Scholar 

  21. Grant, J. E., Odlaug, B. L., & Kim, S. W. (2010). A clinical comparison of pathological skin picking and obsessive compulsive disorder. Comprehensive Psychiatry,51(4), 347–352.

    PubMed  Article  Google Scholar 

  22. Grant, J. E., Stein, D. J., Woods, D. W., & Keuthen, N. J. (2012). Trichotillomania, skin picking & other body-focused repetitive behaviors. Arlington, VA: American Psychiatric Publishing.

    Google Scholar 

  23. Hall, S. S., Hammond, J. L., & Hustyi, K. M. (2013). Examining the relationship between heart rate and problem behavior: A case study of severe skin picking in Prader–Willi syndrome. American Journal on Intellectual and Developmental Disabilities,118(6), 460–474.

    PubMed  Article  Google Scholar 

  24. Hartley, S. L., MacLean, W. E., Butler, M. G., Zarcone, J., & Thompson, T. (2005). Maladaptive behaviors and risk factors among the genetic subtypes of Prader–Willi syndrome. American Journal of Medical Genetics Part A,136(2), 140–145.

    PubMed  PubMed Central  Article  Google Scholar 

  25. Holland, A. J., Whittington, J. E., Butler, J., Webb, T., Boer, H., & Clarke, D. (2003). Behavioural phenotypes associated with specific genetic disorders: Evidence from a population-based study of people with Prader–Willi syndrome. Psychological Medicine,33(1), 141–153.

    PubMed  Article  Google Scholar 

  26. Hustyi, K. M., Hammond, J. L., Rezvani, A. B., & Hall, S. S. (2013). An analysis of the topography, severity, potential sources of reinforcement, and treatments utilized for skin picking in Prader–Willi syndrome. Research in Developmental Disabilities,34(9), 2890–2899.

    PubMed  Article  Google Scholar 

  27. Kushlick, A., Blunden, R., & Cox, G. (1973). A method of rating behaviour characteristics for use in large scale surveys of mental handicap. Psychological Medicine,3(4), 466–478.

    PubMed  Article  Google Scholar 

  28. McAllister, C. J., Whittingtin, J. E., & Holland, A. J. (2011). Development of the eating behaviour in Prader–Willi syndrome: Advances in our understanding. International Journal of Obesity,35, 188–197.

    PubMed  Article  Google Scholar 

  29. Miller, J. L., & Angulo, M. (2014). An open-label pilot study of N-acetylcysteine for skin-picking in Prader–Willi syndrome. American Journal of Medical Genetics Part A,164(2), 421–424.

    Article  Google Scholar 

  30. Morgan, J. R., Storch, E. A., Woods, D. W., Bodzin, D., Lewin, A. B., & Murphy, T. K. (2010). A preliminary analysis of the phenomenology of skin-picking in Prader–Willi syndrome. Child Psychiatry & Human Development,41, 448–463.

    Article  Google Scholar 

  31. Najdowski, A. C., Wallace, M. D., Ellsworth, C. L., MacAleese, A. N., & Cleveland, J. M. (2008). Functional analyses and treatment of precursor behavior. Journal of Applied Behavior Analysis,41(1), 97–105.

    PubMed  PubMed Central  Article  Google Scholar 

  32. Odlaug, B. L., & Grant, J. E. (2008). Clinical characteristics and medical complications of pathological skin picking. General Hospital Psychaitry,30(1), 61–66.

    Article  Google Scholar 

  33. Odlaug, B. L., & Grant, J. E. (2012). Pathological skin picking. In J. E. Grant, D. J. Stein, D. W. Woods, & N. J. Keuthen (Eds.), Trichotillomania, skin picking & other body-focused repetitive behaviors. Arlington, VA: American Psychiatric Publishing.

    Google Scholar 

  34. Oliver, C., McClintock, K., Hall, S., Smith, M., Dagnan, D., & Stenfert-Kroese, B. (2003). Assessing the severity of challenging behaviour: Psychometric properties of the challenging behaviour interview. Journal of Applied Research in Intellectual Disabilities,16, 53–61.

    Article  Google Scholar 

  35. Oliver, C., & Richards, C. (2015). Practitioner review: Self-injurious behaviour in children with developmental delay. Journal of Child Psychology and Psychiatry. https://doi.org/10.1111/jcpp.12425.

    Article  PubMed  Google Scholar 

  36. Oppenheim, A. N. (2005). Questionnaire design, interviewing and attitude measurement: New edition. New York, NY: Continuum.

    Google Scholar 

  37. Prochaska, J. O., & DiClemente, C. C. (1982). Transtheoretical therapy: Toward a more integrative model of change. Psychotherapy Theory, Research & Practice,19(3), 276.

    Article  Google Scholar 

  38. Radstaake, M., Didden, R., Bolio, M., Lang, R., Lancioni, G. E., & Curfs, L. M. (2011). Functional assessment and behavioral treatment of skin picking in a teenage girl with Prader–Willi syndrome. Clinical Case Studies,10, 67–78.

    Article  Google Scholar 

  39. Robb, N., Waller, A., & Woodcock, K. A. (2019). Developing a task switching training game for children with a rare genetic syndrome linked to intellectual disability. Simulation & Gaming, 50(2), 160–179.

    Article  Google Scholar 

  40. Roberts, S., O’Connor, K., & Bélanger, C. (2013). Emotion regulation and other psychological models for body-focused repetitive behaviours. Clinical Psychology Review,33(6), 745–762.

    PubMed  Article  Google Scholar 

  41. Rollnick, S., & Miller, W. R. (1995). What is motivational interviewing? Behavioural and Cognitive Psychotherapy,23(04), 325–334.

    Article  Google Scholar 

  42. Selikowitz, M., Sunman, J., Pendergast, A., & Wright, S. (1990). Fenfluramine in Prader–Willi syndrome: A double blind, placebo controlled trial. Archives of Disease in Childhood,65, 112–114.

    PubMed  PubMed Central  Article  Google Scholar 

  43. Shapira, N. A., Lessig, M. C., Lewis, M. H., Goodman, W. K., & Driscoll, D. J. (2004). Effects of topiramate in adults with Prader–Willi syndrome. American Journal on Mental Retardation,109(4), 301–309.

    PubMed  Article  Google Scholar 

  44. Singh, N. N., Lancioni, G. E., Myers, R. E., Karazsia, B. T., Courtney, T. M., & Nugent, K. (2017). A mindfulness-based intervention for self-management of verbal and physical aggression by adolescents with Prader–Willi syndrome. Developmental Neurorehabilitation,20(5), 253–260.

    PubMed  Article  Google Scholar 

  45. Symons, F. J., Butler, M. G., Sanders, M. D., Feurer, I. D., & Thompson, T. (1999). Self-injurious behavior and Prader–Willi syndrome: Behavioral forms and body locations. American Journal on Mental Retardation,104(3), 260–269.

    PubMed  Article  Google Scholar 

  46. Toogood, S., & Timlin, K. (1996). The functional assessment of challenging behaviour: A comparison of informant-based, experimental and descriptive methods. Journal of Applied Research in Intellectual Disabilities,9(3), 206–222.

    Article  Google Scholar 

  47. Tunnicliffe, P., Woodcock, K., Bull, L., Penhallow, J., & Oliver, C. (2014). Temper outbursts in Prader–Willi syndrome: Causes, behavioural and emotional sequence and responses by carers. Journal of Intellectual Disability Research,58(2), 134–150.

    PubMed  Article  Google Scholar 

  48. Van Ameringen, M., Patterson, B., & Simpson, W. (2014). DSM-5 obsessive-compulsive and related disorders: Clinical implications of new criteria. Depression and Anxiety,31(6), 487–493.

    PubMed  Article  Google Scholar 

  49. Whittington, J. E., Holland, A. J., Webb, T., Butler, J., Clarke, D., & Boer, H. (2004). Cognitive abilities and genotype in a population-based sample of people with Prader–Willi syndrome. Journal of Intellectual Disability Research,48, 172–187.

    PubMed  Article  Google Scholar 

  50. Wigren, M., & Hansen, S. (2005). ADHD symptoms and insistence on sameness in Prader-Willi syndrome. Journal on Intellectual Disability Research,49, 449–456.

    Article  Google Scholar 

  51. Wigren, M., & Heimann, M. (2010). Excessive picking in Prader–Willi syndrome: A pilot study of phenomenological aspects and comorbid symptoms. International Journal of Diasability, Development and Education,48(2), 129–142.

    Article  Google Scholar 

  52. Wilson, D. M., Iwata, B. A., & Bloom, S. E. (2012). Computer-assisted measurement of wound size associated with self-injurious behaviour. Journal of Applied Behavior Analysis,45(4), 797–808.

    PubMed  PubMed Central  Article  Google Scholar 

  53. Woodcock, K., Oliver, C., & Humphreys, G. (2009). Associations between repetitive questioning, resistance to change, temper outbursts and anxiety in Prader–Willi and Fragile-X syndromes. Journal of Intellectual Disability Research,53, 265–278.

    PubMed  Article  Google Scholar 

  54. Ye, L., Bawa, R., & Lippmann, S. (2013). Compulsive skin manipulation treated by topiramate. Journal of child and adolescent psychopharmacology,23(5), 369–370.

    PubMed  Article  Google Scholar 

Download references

Acknowledgments

Thank you to the families who took part making this research possible. Thank you to Emma Shepherd for supporting the research by coding the interviews.

Funding

None.

Author information

Affiliations

Authors

Corresponding authors

Correspondence to L. E. Bull or K. A. Woodcock.

Ethics declarations

Conflict of interest

All authors declare that they have no conflict of interest.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary file1 (DOCX 38 kb)

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Bull, L.E., Oliver, C. & Woodcock, K.A. Skin Picking in People with Prader–Willi Syndrome: Phenomenology and Management. J Autism Dev Disord (2020). https://doi.org/10.1007/s10803-020-04504-5

Download citation

Keywords

  • Prader–Willi syndrome
  • Skin picking
  • Self-injurious behaviour
  • Neurodevelopmental disorder