Skip to main content
SpringerLink
Log in

Prurigo Pigmentosa: Literature Review

  • Review Article
  • Published:
American Journal of Clinical Dermatology Aims and scope Submit manuscript

Abstract

Prurigo pigmentosa, also referred to as Nagashima’s disease, is a rare inflammatory skin condition of unknown etiology. It typically presents as pruritic erythematous papules, papulovesicles, and vesicles appearing in a reticular pattern on the back, chest, or neck. The histological features of prurigo pigmentosa vary according to the stage of the disease. Early-stage disease is characterized by a superficial perivascular infiltrate of neutrophils; spongiosis and necrotic keratinocytes commonly appear in later stages. The etiology of prurigo pigmentosa has yet to be determined. Oral minocycline is usually the first-line therapy for prurigo pigmentosa. However, doxycycline, macrolide antibiotics, and/or dapsone (diaminodiphenyl sulfone) may be indicated for some patients. We describe the key features of prurigo pigmentosa, including the epidemiology, clinical and histologic presentation, differential diagnosis, postulated pathogenesis, and treatment options for this condition.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Böer A, Ackerman AB. Prurigo pigmentosa (Nagashima disease). Textbook and atlas of a distinctive inflammatory disease of the skin. New York: Ardor Scribendi Ltd; 2004. p. 9–39.

    Google Scholar 

  2. Böer A, Misago N, Wolter M, Kiryu H, Wang XD, Ackerman AB. Prurigo pigmentosa: a distinctive inflammatory disease of the skin. Am J Dermatopathol. 2003;25(2):117–29 (PMID:12652193).

    Article  PubMed  Google Scholar 

  3. Joyce AP, Horn TD, Anhalt GJ. Prurigo pigmentosa. Report of a case and review of the literature. Arch Dermatol. 1989;125(11):1551–4 (PMID:2684024).

    Article  CAS  PubMed  Google Scholar 

  4. Asgari M, Daneshpazhooh M, Davatchi CC, Böer A. Prurigo pigmentosa: an underdiagnosed disease in patients of Iranian descent? J Am Acad Dermatol. 2006;55(1):131–6 (PMID:16781307).

    Article  PubMed  Google Scholar 

  5. Cho YT, Liao YH. Prurigo pigmentosa-like persistent papules and plaques in a patient with adult-onset Still’s disease. Acta Derm Venereol. 2014;94(1):102–3 (PMID:23624720).

    Article  PubMed  Google Scholar 

  6. Cota C, Donati P, Amantea A. Prurigo pigmentosa associated with an atopic diathesis in a 13-year-old girl. Pediatr Dermatol. 2007;24(3):277–9 (PMID:17542880).

    Article  PubMed  Google Scholar 

  7. Erbagci Z. Prurigo pigmentosa in association with Helicobacter pylori infection in a Caucasian Turkish woman. Acta Derm Venereol. 2002;82(4):302–3 (PMID:12361140).

    Article  PubMed  Google Scholar 

  8. Togawa Y, Shinkai H, Utani A. Prurigo pigmentosa in a patient with primary biliary cirrhosis and Sjögren syndrome. J Dermatol. 2004;31(10):815–9 (PMID:15672710).

    Article  PubMed  Google Scholar 

  9. Nagashima M, Ohshiro A, Shimizu N. A peculiar pruriginous dermatosis with gross reticular pigmentation. Jpn J Dermatol. 1971;81:38–9 (no PMID).

    Google Scholar 

  10. Nagashima M. Prurigo pigmentosa—clinical observations of our 14 cases. J Dermatol. 1978;5(2):61–7 (PMID:353107).

    Article  CAS  PubMed  Google Scholar 

  11. Abbass M, Abiad F, Abbas O. Prurigo pigmentosa after bariatric surgery. JAMA Dermatol. 2015;151(7):796–97 (PMID:25853788).

    Article  PubMed  Google Scholar 

  12. Shin JW, Lee SY, Lee JS, Whang KU, Park YL, Lee HK. Prurigo pigmentosa in Korea: clinicopathological study. Int J Dermatol. 2012;51(2):152–7 (PMID:22250622).

    Article  PubMed  Google Scholar 

  13. Whang T, Kirkorian AY, Krishtul A, Phelps R, Shim-Chang H. Prurigo pigmentosa: report of two cases in the United States and review of the literature. Dermatol Online J. 2011;17(12):2 (PMID:22233738).

    PubMed  Google Scholar 

  14. Teraki Y, Nishikawa T. Skin diseases described in Japan 2004. J Dtsch Dermatol Ges. 2005;3(1):9–25 (PMID:16353745).

    Article  PubMed  Google Scholar 

  15. Böer A, Asgari M. Prurigo pigmentosa: an underdiagnosed disease? Indian J Dermatol Venereol Leprol. 2006;72(6):405–9 (PMID:17179613).

    Article  PubMed  Google Scholar 

  16. El-Darouti MA. Chapter 76—recurrent pruritic eruptions resolving with reticulate pigmentation. In: Challenging cases in dermatology, 1st edition. Springer: Dodrecht; 2012. p. 539–43.

  17. Scheinfeld N. Confluent and reticulated papillomatosis: a review of the literature. Am J Clin Dermatol. 2006;7(5):305–13 (PMID:17007541).

    Article  PubMed  Google Scholar 

  18. Riahi RR, Cohen PR, Robinson FW, Gray JM. Erythema ab igne mimicking livedo reticularis. Int J Dermatol. 2010;49(11):1314–7 (PMID:20964656).

    Article  PubMed  Google Scholar 

  19. Miller K, Hunt R, Chu J, Meehan S, Stein J. Erythema ab igne. Dermatol Online J. 2011;17(10):28 (PMID:22031654).

    PubMed  Google Scholar 

  20. Riahi RR, Cohen PR. What caused this hyperpigmented reticulated rash on this man’s back? (Erythema ab igne). Dermatologist. 2013;21:47–50 (no PMID).

    Google Scholar 

  21. Nakada T, Sueki H, Iijima M. Prurigo pigmentosa (Nagashima) associated with anorexia nervosa. Clin Exp Dermatol. 1998;23(1):25–7 (PMID:9667105).

    Article  CAS  PubMed  Google Scholar 

  22. Kobayashi T, Kawada A, Hiruma M, Ishibashi A, Aoki A. Prurigo pigmentosa, ketonemia and diabetes mellitus. Dermatology. 1996;192(1):78–80 (PMID:8832962).

    Article  CAS  PubMed  Google Scholar 

  23. Oh YJ, Lee MH. Prurigo pigmentosa: a clincopathologic study of 16 cases. J Eur Acad Dermatol Venereol. 2012;26(9):1149–53 (PMID:21929552).

    Article  CAS  PubMed  Google Scholar 

  24. Kim MH, Choi YW, Choi HY, Myung KB. Prurigo pigmentosa from contact allergy to chrome in detergent. Contact Dermatitis. 2001;44(5):289–92 (PMID:11298695).

    Article  CAS  PubMed  Google Scholar 

  25. Ekmekci TR, Altunay IK, Koslu A. Prurigo pigmentosa treated with doxycycline. Dermatol Online J. 2006;12(1):9 (PMID:16638377).

    PubMed  Google Scholar 

  26. Yazawa N, Ihn H, Yamane K, Etoh T, Tamaki K. The successful treatment of prurigo pigmentosa with macrolide antibiotics. Dermatology. 2001;202(1):67–9 (PMID:11244235).

    Article  CAS  PubMed  Google Scholar 

  27. Kwon HJ, Kim MY, Kim HO, Park YM. Two cases of prurigo pigmentosa in atopic patients. J Dermatol. 2006;33(8):579–82 (PMID:16923144).

    Article  PubMed  Google Scholar 

  28. Atasoy M, Timur H, Arslan R, Ozdemir S, Gursan N, Erdem T. Prurigo pigmentosa in a patient with nickel sensitivity. J Eur Acad Dermatol Venereol. 2009;23(2):228–30 (PMID:18498337).

    Article  CAS  PubMed  Google Scholar 

  29. Tomaru K, Nagai Y, Ohyama N, Hasegawa M, Endo Y, Tamura A, Ishikawa O. Adult-onset Still’s disease associated with prurigo pigmentosa-like skin eruption. J Dermatol. 2006;33(1):55–8 (PMID:16469087).

    Article  PubMed  Google Scholar 

  30. Missall TA, Pruden S, Nelson C, Fohn L, Vidal CI, Hurley MY. Identification of Helicobacter pylori in skin biopsy of prurigo pigmentosa. Am J Dermatopathol. 2012;34(4):446–8 (PMID:22197863).

    Article  PubMed  Google Scholar 

  31. Chao LL, Lu CF, Shih CM. Molecular detection and genetic identification of Borrelia garinii and Borrelia afzelii from patients presenting with a rare skin manifestation of prurigo pigmentosa in Taiwan. Int J Infect Dis. 2013;17(12):e1141–7 (PMID:24103333).

    Article  CAS  PubMed  Google Scholar 

  32. Rodríguez-Díaz E, Blanco S, Alvarez-Cuesta C, Galache C, Barrio A, Corte G. Prurigo pigmentosa. Actas Dermosifiliogr. 2005;96(7):441–5 (PMID:16476271).

    Article  PubMed  Google Scholar 

  33. De Francesco V, Quinkenstein E, Mariuzzi L, Frattasio A, Pillon B, Patrone P. Bullous prurigo pigmentosa. Eur J Dermatol. 2006;16(2):184–6 (PMID:16581574).

    PubMed  Google Scholar 

  34. Baykal C, Buyukbabani N, Akinturk S, Saglik E. Prurigo pigmentosa: not an uncommon disease in the Turkish population. Int J Dermatol. 2006;45(10):1164–8 (PMID:17040430).

    Article  PubMed  Google Scholar 

  35. Shannon JF, Weedon D, Sharkey MP. Prurigo pigmentosa. Australas J Dermatol. 2006;47(4):289–90 (PMID:17034475).

    Article  PubMed  Google Scholar 

  36. Akoglu G, Boztepe G, Karaduman A. Prurigo pigmentosa successfully treated with low-dose isotretinoin. Dermatology. 2006;213(4):331–3 (PMID:17135740).

    Article  PubMed  Google Scholar 

  37. Kim SK, Kang HY, Lee ES. Bullous prurigo pigmentosa. Int J Dermatol. 2007;46(8):888–90 (PMID:17651184).

    Article  PubMed  Google Scholar 

  38. Park HY, Hong SP, Ahn SY, Ji JH, Choi EH, Jeon SY. Antinuclear antibodies in patients with prurigo pigmentosa: a linkage or coincidence? Dermatology. 2009;218(1):90–1 (PMID:18832815).

    Article  PubMed  Google Scholar 

  39. Chiam LY, Goh BK, Lim KS, Ng SK. Prurigo pigmentosa: a report of two cases that responded to minocycline. Clin Exp Dermatol. 2009;34(8):e584–6 (PMID:19489866).

    Article  CAS  PubMed  Google Scholar 

  40. Marín PR, Ochoa RA, Cudós ES, Muruzábal UF, Jiménez MC, Díaz de Arcaya CV. Pruritic reticular eruption on the chest of a 24-year-old woman—quiz case. Diagnosis: prurigo pigmentosa (PP). Arch Dermatol. 2010;146(1):81–6 (PMID:20083701).

    Article  PubMed  Google Scholar 

  41. Lin SH, Ho JC, Cheng YW, Huang PH, Wang CY. Prurigo pigmentosa: a clinical and histopathologic study of 11 cases. Chang Gung Med J. 2010;33(2):157–63 (PMID:20438668).

    PubMed  Google Scholar 

  42. Wu CY. Pruritic brownish confluent reticulated papules over the trunk of a 30-year-old woman. Clin Exp Dermatol. 2010;35(3):335–6 (PMID:20500205).

    Article  PubMed  Google Scholar 

  43. Schepis C, Siragusa M, Cavallari V. An intriguing case of prurigo pigmentosa in a Sicilian young patient. G Ital Dermatol Venereol. 2010;145(4):551–4 (PMID:20823798).

    CAS  PubMed  Google Scholar 

  44. Lu PH, Hui RC, Yang LC, Yang CH, Chung WH. Prurigo pigmentosa: a clinicopathological study and analysis of associated factors. Int J Dermatol. 2011;50(1):36–43 (PMID:21182500).

    Article  PubMed  Google Scholar 

  45. Kokol R, Miljkovic J, El Shabrawi-Caelen L. Prurigo pigmentosa. J Dtsch Dermatol Ges. 2011;9(10):867–8 (PMID:21801307).

    PubMed  Google Scholar 

  46. Mok YJ, Lim KS, Tey HL. Doxycycline—an emerging therapy for prurigo pigmentosa. J Eur Acad Dermatol Venereol. 2012;26(4):526–7 (PMID:21521380).

    Article  CAS  PubMed  Google Scholar 

  47. Kim JK, Chung WK, Chang SE, Ko JY, Lee JH, Won CH, Lee MW, Choi JH, Moon KC. Prurigo pigmentosa: clinicopathological study and analysis of 50 cases in Korea. J Dermatol. 2012;39(11):891–7 (PMID:22901017).

    Article  PubMed  Google Scholar 

  48. Jonak C, Riedl E. An itchy rash in a young Caucasian woman. Dermatol Pract Concept. 2012;2(3):203a04 (PMID:23785606).

    Article  PubMed  Google Scholar 

  49. Ilkovitch D, Patton TJ. Is prurigo pigmentosa an inflammatory version of confluent and reticulated papillomatosis? J Am Acad Dermatol. 2013;69(4):e193–5 (PMID:24034398).

    Article  PubMed  Google Scholar 

  50. Torrelo A, Azorín D, Noguera L, Hernández-Martín A, Happle R, Requena L. Segmental prurigo pigmentosa. Pediatr Dermatol. 2014;31(4):523–5 (PMID:24846714).

    Article  PubMed  Google Scholar 

  51. Hijazi M, Kehdy J, Kibbi AG, Ghosn S. Prurigo pigmentosa: a clinicopathologic study of 4 cases from the middle East. Am J Dermatopathol. 2014;36(10):800–6 (PMID:25079199).

    Article  PubMed  Google Scholar 

  52. Gironi LC, Farinelli P, Giacalone A, Colombo E. The efficacy of minocycline in inflammatory dermatoses: a case of prurigo pigmentosa of prepubescent onset in Western world. Dermatol Ther. 2015;28(4):239–42 (PMID:25754558).

    Article  PubMed  Google Scholar 

  53. Mitsuhashi Y, Suzuki N, Kawaguchi M, Kondo S. Prurigo pigmentosa on a patient with soft-drink ketosis. J Dermatol. 2005;32(9):767–8 (PMID:16361724).

    Article  PubMed  Google Scholar 

  54. Choi JR, Kim JK, Won CH, Lee MW, Oh ES, Chang S. Prurigo pigmentosa treated with Jessner’s peel and irradiation with an 830-nm light-emitting diode. J Dermatol. 2012;39(5):493–6 (PMID:22077395).

    Article  PubMed  Google Scholar 

  55. Michaels JD, Hoss E, DiCaudo DJ, Price H. Prurigo pigmentosa after a strict ketogenic diet. Pediatr Dermatol. 2015;32(2):248–51 (PMID:24372546).

    Article  PubMed  Google Scholar 

  56. Leone L, Colato C, Girolomoni G. Prurigo pigmentosa in a pregnant woman. Int J Gynaecol Obstet. 2007;98(3):261–2 (PMID:17532321).

    Article  CAS  PubMed  Google Scholar 

  57. Jang MS, Baek JW, Kang DY, Kang JS, Kim ST, Suh KS. Successful treatment with narrowband UVB phototherapy in prurigo pigmentosa associated with pregnancy. Eur J Dermatol. 2011;21(4):634–5 (PMID:21697051).

    PubMed  Google Scholar 

  58. Hanami Y, Yamamoto T. Bullous prurigo pigmentosa in a pregnant woman with hyperemesis gravidarum. J Dermatol. 2015;42(4):436–7 (PMID:25728465).

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. University of Nevada School of Medicine, 1060 Wiegand Road, Encinitas, CA, 92024, USA

    Bryce D. Beutler

  2. Department of Dermatology, University of California San Diego, San Diego, CA, USA

    Philip R. Cohen & Robert A. Lee

  3. Dermatopathology, University of California San Diego, San Diego, CA, USA

    Robert A. Lee

Authors
  1. Bryce D. Beutler
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Philip R. Cohen
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Robert A. Lee
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Bryce D. Beutler.

Ethics declarations

Funding

No funding was received for the preparation of this review.

Conflict of interest

Bryce D. Beutler, Philip R. Cohen, and Robert A. Lee have no conflicts of interest.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Beutler, B.D., Cohen, P.R. & Lee, R.A. Prurigo Pigmentosa: Literature Review. Am J Clin Dermatol 16, 533–543 (2015). https://doi.org/10.1007/s40257-015-0154-4

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40257-015-0154-4

Keywords

Search

Navigation