Skip to main content
SpringerLink
Log in

Cutaneous manifestations associated with adult-onset Still’s disease: important diagnostic values

  • Review
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Adult-onset Still’s disease (AOSD) is a systemic inflammatory condition, characterized by a high spiking fever, leukocytosis with neutrophilia, arthralgia, and skin rash. Typical skin rash is an evanescent, salmon-pink erythema predominantly involving extremities, which is included as one of the diagnostic criteria; however, recent findings show that not only typical evanescent rash but also various skin lesions are associated with AOSD. The representative characteristic skin lesion among the non-classical skin rash is called persistent pruritic papules and plaques, which presents erythematous, slightly scaly papules with linear configuration on the trunk. Interestingly, persistent pruritic papules and plaques show unique histological features such as peculiar, distinctive distribution of dyskeratotic keratinocytes in the cornified layers as well as in the epidermis. Other non-classical skin lesions include urticaria. Current insights suggest that several inflammatory cytokines such as interleukin-1 (IL-1), IL-6, IL-18, interferon-γ (IFN-γ) and tumor necrosis factor-α (TNF-α) play a pathogenic role in AOSD. In particular, IL-18 is suggested to play a crucial role in activating macrophages, favoring Th1 type cytokine production. IL-18 induces IFN-γ, IL-17, and TNF-α, which may play an important pathogenic role in AOSD. It is important to recognize the common and/or uncommon skin conditions of AOSD for early correct diagnosis. In this review, various skin lesions are introduced, and the complication with histiocytic necrotizing lymphadenitis (Kikuchi disease) is further discussed.

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
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7

Similar content being viewed by others

References

  1. Bagnari V, Colina M, Ciancio G, Govoni M, Trotta F (2010) Adult-onset Still’s disease. Rheumatol Int 30:855–862

    Article  PubMed  CAS  Google Scholar 

  2. Efthimiou P, Kontzias A, Ward CM, Ogden NS (2007) Adult-onset Still’s disease: can recent advances in our understanding of its pathogenesis lead to target therapy? Nat Rev Rheumatol 3:328–335

    CAS  Google Scholar 

  3. Fautrel B (2008) Adult-onset Still’s disease. Best Pract Res Clin Rheumatol 22:773–792

    Article  PubMed  Google Scholar 

  4. Fautrel B, Le Moël G, Saint-Marcoux B et al (2001) Diagnostic value of ferritin and glycosylated ferritin in adult onset Still’s disease. J Rheumatol 28:322–329

    PubMed  CAS  Google Scholar 

  5. Cush JJ, Medsger TA Jr, Christy WC, Herbert DC, Cooperstein LA (1987) Adult-onset Still’s disease: clinical course and outcome. Arthritis Rheum 30:186–194

    Article  PubMed  CAS  Google Scholar 

  6. Yamaguchi M, Ohta A, Tsunematsu T et al (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19:424–431

    PubMed  CAS  Google Scholar 

  7. Fautrel B, Zing E, Golmard JL et al (2002) Proposal for a new set of classification criteria for adult-onset Still disease. Medicine (Baltimore) 81:194–200

    Article  Google Scholar 

  8. Hoshino T, Ohta A, Yang D, Kawamoto M, Kikuchi M, Inoue Y et al (1998) Elevated serum interleukin 6, interferon-γ, and tumor necrosis factor-α levels in patients with adult Still’s disease. J Rheumatol 25:396–398

    PubMed  CAS  Google Scholar 

  9. Fujii T, Nojima T, Yasuoka H, Satoh S, Nakamura K, Kuwana M et al (2001) Cytokine and immunogenic profiles in Japanese patients with adult Still’s disease: association with chronic articular disease. Rheumatology 40:1398–1404

    Article  PubMed  CAS  Google Scholar 

  10. Choi JH, Suh CH, Lee YM, Suh YJ, Lee SK, Kim SS et al (2003) Serum cytokine profiles in patients with adult onset Still’s disease. J Rheumatol 30:2422–2427

    PubMed  CAS  Google Scholar 

  11. Kawaguchi Y, Terajima H, Harigai M, Hara M, Kamatani N (2001) Interleukin-18 as a novel diagnostic marker and indicator of disease severity in adult-onset Still’s disease. Arthritis Rheum 44:1716–1718

    Article  PubMed  CAS  Google Scholar 

  12. Kawashima M, Yamamura M, Taniai M, Yamaguchi H, Tanimoto T et al (2001) Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still’s disease. Arthritis Rheum 44:550–560

    Article  PubMed  CAS  Google Scholar 

  13. Chen DY, Lan JL, Lin FJ, Hsieh TY (2004) Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still’s disease. J Rheumatol 31:2189–2198

    PubMed  CAS  Google Scholar 

  14. Harrington LE, Mangan PR, Weaver CT (2006) Expanding the effector CD4 T-cell repertoire: the Th17 lineage. Curr Opin Immunol 18:340–356

    Article  Google Scholar 

  15. Carroll HP, Paunovic V, Gadina M (2008) Crossed signals: the role of interleukin-15 and -18 in autoimmunity. Rheumatology 47:1269–1277

    Article  PubMed  CAS  Google Scholar 

  16. Yamamoto T, Nishioka K (2006) Flagellate erythema. Int J Dermatol 45:627–631

    Article  PubMed  Google Scholar 

  17. Kaur S, Bambery P, Dhar S (1994) Persistent dermal plaque lesions in adult onset Still’s disease. Dermatology 188:241–242

    Article  PubMed  CAS  Google Scholar 

  18. Lübbe J, Hofer M, Chavaz P, Saurat JH, Borradori L (1999) Adult-onset Still’s disease with persistent plaques. Br J Dermatol 141:710–713

    Article  PubMed  Google Scholar 

  19. Suzuki K, Kimura Y, Aoki M, Takezaki S, Tsuchida T, Takano T, Kawana S (2001) Persistent plaques and linear pigmentation in adult-onset Still’s disease. Dermatology 202:333–335

    Google Scholar 

  20. Fujii K, Konishi K, Kanno Y, Ohgou N (2003) Persistent generalized erythema in adult-onset Still’s disease. Int J Dermatol 42:824–825

    Google Scholar 

  21. Thien Huong NT, Pitche P, Minh Hoa T, Laurent R (2005) Persistent pigmented plaques in adult-onset Still’s disease. Ann Dermatol Venereol 132:693–696

    Article  PubMed  Google Scholar 

  22. Lee JY-Y, Yang C-C, Hsu MM-L (2005) Histopathology of persistent papules and plaques in adult-onset Still’s disease. J Am Acad Dermatol 52:1003–1008

    Article  PubMed  Google Scholar 

  23. Wolgamot G, Yoo J, Hurst S, Gardner G, Olerud J, Argenyi Z (2007) Unique histopathologic findings in a patient with adult-onset Still’s disease. Am J Dermatopathol 49:194–196

    Google Scholar 

  24. Fortna RR, Gudjonsson JE, Seidel G, DiCostanzo D, Jacobson M, Kopelman M, Patel RM (2010) Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult-onset and juvenile Still’s disease. J Cutan Pathol 37:932–937

    Article  PubMed  Google Scholar 

  25. Yang C–C, Lee JY-Y, Liu M-F, Ho C-L (2006) Adult-onset Still’s disease with persistent skin eruption and fatal respiratory failure in a Taiwanese woman. Eur J Dermatol 16:593–594

    PubMed  Google Scholar 

  26. Affleck AG, Littlewood SM (2005) Adult-onset Still’s disease with atypical cutaneous features. J Eur Acad Dermatol Venereol 19:360–363

    Article  PubMed  CAS  Google Scholar 

  27. Setterfield JF, Hughes GRV, Kobza-Black A (1998) Urticaria as a presentation of adult Still’s disease. Br J Dermatol 138:906–908

    Article  PubMed  CAS  Google Scholar 

  28. Salaffi F, Filosa G, Bugatti L, Maestrini MD (2000) Urticaria as a presenting manifestation of adult-onset Still’s disease. Clin Rheumatol 19:389–391

    Article  PubMed  CAS  Google Scholar 

  29. Criado RF, Criado PR, Vasconcellos C, Szajubok JC, Michalany NS, Kadune BV et al (2006) Urticaria as a cutaneous sign of adult-onset Still’s disease. J Cutan Med Surg 10:99–103

    PubMed  Google Scholar 

  30. Criado PR, de Carvalho JF, Ayabe LA, Brandt HR, Romiti R, Maruta CW (2011) Urticaria and dermographism in patients with adult-onset Still’s disease. Rheumatol Int [Epub ahead of print]

  31. Lee JB, Kim JW, Lee SS, Kim SJ, Won YH, Lee SC (2002) Adult-onset Still’s disease with vesiculopustules on the hands and feet. J Korean Med Sci 17:852–853

    PubMed  Google Scholar 

  32. Bachmeyer C, Blum L, Petitjean B, Kemiche F, Roujeau JC, Pertuiset E (2006) Vesiculopustules in adult-onset Still’s disease. J Am Acad Dermatol 54:S247–S248

    Article  PubMed  Google Scholar 

  33. Phillips WG, Weller R, Handfield-Jones SE, Kobza-Black A (1994) Adult Still’s disease. Br J Dermatol 130:511–513

    Article  PubMed  CAS  Google Scholar 

  34. Tomaru K, Nagai Y, Ohyama N, Hasegawa M, Endo Y, Tamura A, Ishikawa O (2006) Adult-onset Still’s disease with prurigo pigmentosa-like skin eruption. J Dermatol 33:55–58

    Article  PubMed  Google Scholar 

  35. Soy M (2004) A case of adult-onset Still’s disease presenting as angioedema. Clin Rheumatol 23:92

    Article  PubMed  Google Scholar 

  36. Mylona E, Vadala C, Papadakos V, Loverdos D, Samarkos M, Skoutelis A (2009) Cutaneous polyarteritis nodosa in adult onset Still’s disease. Eur J Dermatol 19:621–622

    PubMed  Google Scholar 

  37. Elinav H, Maly A, Ilan Y, Rubinow A, Naparstek Y, Amital H (2004) The coexistence of Sweet’s syndrome and Still’s disease: is it merely a coincidence? J Am Acad Dermatol 50:S90–S92

    Article  PubMed  Google Scholar 

  38. Perez C, Montes M, Gallego M, Loza E (2001) Atypical presentation of adult Still’s disease with generalized rash and hyperferritinaemia. Br J Dermatol 145:187–188

    Article  PubMed  CAS  Google Scholar 

  39. Ohta A, Matsumoto Y, Ohta T, Kaneoka H, Yamaguchi M (1988) Still’s disease associated with necrotizing lymphadenitis (Kikuchi’s disease): report of 3 cases. J Rheumatol 15:981–983

    PubMed  CAS  Google Scholar 

  40. Lyberatos C (1990) Two more cases of Still’s disease and Kikuchi’s. J Rheumatol 17:568–569

    PubMed  CAS  Google Scholar 

  41. Cousin F, Grézard P, Roth B, Balme B, Grégoire-Bardel M, Perrot H (1999) Kikuchi disease associated with Still disease. Int J Dermatol 38:464–473

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

The author has declared that there is no conflicts of interest.

Author information

Authors and Affiliations

  1. Department of Dermatology, Fukushima Medical University, Hikarigaoka 1, Fukushima, 960-1295, Japan

    Toshiyuki Yamamoto

Authors
  1. Toshiyuki Yamamoto
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Toshiyuki Yamamoto.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Yamamoto, T. Cutaneous manifestations associated with adult-onset Still’s disease: important diagnostic values. Rheumatol Int 32, 2233–2237 (2012). https://doi.org/10.1007/s00296-011-2330-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-011-2330-z

Keywords

Search

Navigation