Pustular Psoriasis

  • Sebastian Bernardo
  • Mark LebwohlEmail author


Psoriasis is a polygenic, chronic inflammatory skin disease linked to dysregulation of the immune system that classically presents as sharply demarcated erythematous plaques with silvery scale [1, 2]. In some patients, the condition can manifest as a generalized pustular variant that can follow a severe, potentially life-threatening course that demands urgent dermatological evaluation and treatment [1]. While pustules may be a part of the clinical picture in both pustular psoriasis and acne vulgaris, comedones are classically absent in the former. Further, pustular psoriasis commonly spares the face, even in its generalized form. Unlike severe acne, patients presenting with pustular psoriasis may be toxic appearing on physical exam and be found to have hematological abnormalities and electrolyte imbalances that represent a true dermatologic emergency.


Pustular Psoriasis Acute Generalize Exanthematous Pustulosis Severe Acne Palmoplantar Pustulosis Chronic Inflammatory Skin Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Roosevelt L, Emer J. Pustular psoriasis: a practical review of the literature. Psoriasis Forum. 2011;17(1):33–41.Google Scholar
  2. 2.
    Van de Kerkhof PCM. Psoriasis. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. New York, NY: Mosby [Elsevier]; 2003. p. 125–46.Google Scholar
  3. 3.
    National Psoriasis Foundation—Psoriasis statistics, prevalence, severity, age of onset and psoriasis research. The National Psoriasis Foundation Website. Accessed 29 Sept 2011.
  4. 4.
    Stern RS, Nijsten T, Feldman SR, Margolis DJ, Rolstad T. Psoriasis is common, carries a substantial burden even when not extensive, and is associated with widespread treatment dissatisfaction. J Investig Dermatol Symp Proc. 2004 Mar;9(2):136–9.PubMedCrossRefGoogle Scholar
  5. 5.
    Zelickson BD, Muller SA. Generalized pustular psoriasis: a review of 63 cases. Arch Dermatol. 1991;127:1339–45.PubMedCrossRefGoogle Scholar
  6. 6.
    de Oliveira ST, Maragno L, Arnone M, Fonseca Takahashi MD, Romiti R. Generalized pustular psoriasis in childhood. Pediatr Dermatol. 2010;27:349–54.PubMedCrossRefGoogle Scholar
  7. 7.
    Liao PB, Rubinson R, Howard R, Sanchez G, Frieden IJ. Annular pustular psoriasis—most common form of pustular psoriasis in children: report of three cases and review of the literature. Pediatr Dermatol. 2002;19:19–25.PubMedCrossRefGoogle Scholar
  8. 8.
    Gelfand JM, Stern RS, Nijsten T, et al. The prevalence of psoriasis in african americans: results from a population-based study. J Am Acad Dermatol. 2005;52:23–6.PubMedCrossRefGoogle Scholar
  9. 9.
    Visual Dx—adult rash: psoriasis, pustular. Visual Dx website. Accessed 12 Sept 2011.
  10. 10.
    Gudjonsson JE, Elder JT. Psoriasis. In: Fitzpatrick TB, Wolff K, editors. Fitzpatrick’s dermatology in general medicine. 7th ed. New York, NY: McGraw-Hill; 2008. p. 169–93.Google Scholar
  11. 11.
    Schon MP, Boehncke WH. Psoriasis. N Engl J Med. 2005;352:1899–912.PubMedCrossRefGoogle Scholar
  12. 12.
    Benoit S, Toksoy A, Bröcker EB, Gillitzer R, Goebeler M. Treatment of recalcitrant pustular psoriasis with infliximab: effective reduction of chemokine expression. Br J Dermatol. 2004;150(5):1009–12.PubMedCrossRefGoogle Scholar
  13. 13.
    Yamamoto M, Tada Y, Asahina A, et al. Severe generalized pustular psoriasis accompanied by bullae formation with increased serum vascular endothelial growth factor level. J Dermatol. 2012;39(2):183–5. 2011 July 18. [Epub ahead of print].PubMedCrossRefGoogle Scholar
  14. 14.
    Marrkchi S, Guigue P, Renshaw BR, et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med. 2011;365(7):620–8.CrossRefGoogle Scholar
  15. 15.
    Onoufriadis A, Simpson MA, Pink AE, et al. Mutations in Il36RN/Il1F5 are associated with the severe episodic inflammatory skin disease known as generalized pustular psoriasis. Am J Hum Genet. 2011;89(3):432–7.PubMedCrossRefGoogle Scholar
  16. 16.
    Umezawa Y, Ozawa A, Kawasima T, et al. Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity. Arch Dermatol Res. 2003;295(1):S43–54.PubMedGoogle Scholar
  17. 17.
    Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, editors. Dermatology in general medicine. 6th ed. New York, NY: McGraw Hill; 2003.Google Scholar
  18. 18.
    Brenner M, Molin S, Ruebsam K, Weisenseel P, Ruzicka T, Prinz JC. Generalized pustular psoriasis induced by systemic glucocorticosteroids: four cases and recommendations for treatment. Br J Dermatol. 2009;161(4):964–6.PubMedCrossRefGoogle Scholar
  19. 19.
    Ryan T, Baker H. Systemic corticosteroids and folic acid antagonists in the treatment of generalized pustular psoriasis evaluation and prognosis based on the study of 104 cases. Br J Dermatol. 1969;81:134–45.PubMedCrossRefGoogle Scholar
  20. 20.
    Rongioletti F, Fiorucci C, Parodi A. Psoriasis induced or aggravated by drugs. J Rhematol Suppl. 2009;83:59–61.CrossRefGoogle Scholar
  21. 21.
    Park JJ, Lee SC. A case of tumor necrosis factor-α inhibitors-induced pustular psoriasis. Ann Dermatol. 2010;22:212–5.PubMedCrossRefGoogle Scholar
  22. 22.
    Wenk KS, Claros JM, Ehrlich A. Flare of pustular psoriasis after initiating ustekinumab therapy. J Dermatolog Treat. 2012;23(3):212–4. 2011 Jan 22 [Epub ahead of print].PubMedCrossRefGoogle Scholar
  23. 23.
    Pourciau C, Shwayder T. Occurrence of pustular psoriasis after treatment of Crohn disease with infliximab. Pediatr Dermatol. 2010;27(5):539–40.PubMedCrossRefGoogle Scholar
  24. 24.
    Collamer AN, Guerrero KT, Henning JS, Battafarano DF. Psoriatic skin lesions induced by tumor necrosis factor antagonist therapy: a literature review and potential mechanisms of action. Arthritis Rheum. 2008;59(7):996–1001.PubMedCrossRefGoogle Scholar
  25. 25.
    Shmidt E, Wetter DA, Ferguson SB, Pittelkow MR. Psoriasis and palmoplantar pustulosis associated with tumor necrosis factor-α inhibitors: The Mayo Clinic experience, 1998 to 2010. J Am Acad Dermatol. 2012;67(5):e179–85. 2011 Jul 11 [Epub ahead of print].PubMedCrossRefGoogle Scholar
  26. 26.
    Gudjonsson JE, Thorarinsson AM, Sigurgeirsson B, Kristinsson KG, Valdimarsson H. Streptococcal throat infections and exacerbation of chronic plaque psoriasis: a prospective study. Br J Dermatol. 2003;149:530–4.PubMedCrossRefGoogle Scholar
  27. 27.
    Prystowsky JH, Cohen PR. Pustular and erytherodermic psoriasis. Psoriasis. 1995;13:757–70.Google Scholar
  28. 28.
    Katz M, Seidenbaum M, Weinrauch L. Penicillin-induced generalized pustular psoriasis. J Am Acad Dermatol. 1987;17:918–20.PubMedCrossRefGoogle Scholar
  29. 29.
    Fitzpatrick TB, Johnson RA, Wolff K, Suurmond D, editors. Color atlas and synopsis of clinical dermatology common and serious diseases. New York, NY: McGraw-Hill; 2001.Google Scholar
  30. 30.
    Maize JC, Burgdorf WHC, Hurt MA, et al. Cutaneous pathology. 1st ed. Philadelphia, PA: Churchill Livingstone; 1998.Google Scholar
  31. 31.
    Barnhill RL, Crowson AN, Magro CM, Piepkorn MW. Dermatopathology. 3rd ed. China: McGraw-Hill; 2010. p. 66–7.Google Scholar
  32. 32.
    Hazarika D. Generalized pustular psoriasis of pregnancy successfully treated with cyclosporine. Indian J Dermatol Venereol Leprol. 2009;75(6):638–41.PubMedCrossRefGoogle Scholar
  33. 33.
    Puig L, Barco D, Vilarrasa E, Alomar A. Treatment of acrodermatitis continua of hallopeau with TNF-blocking agents: case report and review. Dermatology. 2010;220(2):154–8.PubMedCrossRefGoogle Scholar
  34. 34.
    Zelickson BD, Muller SA. Generalized pustular psoriasis in childhood: report of thirteen cases. J Am Acad Dermatol. 1991;24:186–94.PubMedCrossRefGoogle Scholar
  35. 35.
    Viguier M, Allez M, Zagdanski AM, et al. High frequency of cholestasis in generalized pustular psoriasis: evidence for neutrophilic involvement of the biliary tract. Hepatology. 2004;40:452–8.PubMedCrossRefGoogle Scholar
  36. 36.
    Borges-Costa J, Silva R, Goncalves L, et al. Clinical and laboratory features in acute generalized pustular psoriasis. Am J Clin Dermatol. 2011;12(4):271–6.PubMedCrossRefGoogle Scholar
  37. 37.
    Lebwohl MG, Heymann WR, Berth-Jones J, Coulson I. Treatment of skin disease. 2nd ed. China: Mosby-Elsevier; 2006.Google Scholar
  38. 38.
    Sofen HL, Moy RL, Lowe NJ. Treatment of generalised pustular psoriasis with isotretinoin. Lancet. 1984;7:40.CrossRefGoogle Scholar
  39. 39.
    Ozawa A, Ohkido M, Haruki Y, et al. Treatments of generalized pustular psoriasis: a multicenter study in Japan. J Dermatol. 1999;26:141–9.PubMedGoogle Scholar
  40. 40.
    Gollnick HPM. Oral retinoids—efficacy and toxicity in psoriasis. Br J Dermatol. 1996;135(49):6–17.PubMedCrossRefGoogle Scholar
  41. 41.
    Dogra S, Kaur I. Childhood psoriasis. Indian J Dermatol Venereol Leprol. 2010;76(4):357–65.PubMedCrossRefGoogle Scholar
  42. 42.
    Kalb RE, Strober B, Weinstein G, Lebwohl M. Methotrexate and psoriasis: 2009 national psoriasis foundation consensus conference. J Am Acad Dermatol. 2009;60(5):824–37.PubMedCrossRefGoogle Scholar
  43. 43.
    Meinardi MM, Westerhof W, Bos JD. Generalized pustular psoriasis (von zumbusch) responding to cyclosporine A. Br J Dermatol. 1987;116(2):269–70.PubMedCrossRefGoogle Scholar
  44. 44.
    Rosmarin DM, Lebwohl M, Elewski BE, Gottlieb AB. Cyclosporine and psoriasis: 2008 national psoriasis foundation consensus conference. J Am Acad Dermatol. 2010;62(5):838–53.PubMedCrossRefGoogle Scholar
  45. 45.
    Dogra S, Kumaran MS, Handa S, Kanwar AJ. Methotrexate for generalized pustular psoriasis in a 2-year-old child. Pediatr Dermatol. 2005;22(1):85–6.PubMedCrossRefGoogle Scholar
  46. 46.
    Maza A, Montaudie H, Sbidian E, et al. Oral cyclosporine in psoriasis: a systematic review on treatment modalities, risk of kidney toxicity and evidence for use in non-plaque psoriasis. J Eur Acad Dermatol Venereol. 2011;25(2):19–27.PubMedCrossRefGoogle Scholar
  47. 47.
    Paul C, Gallini A, Maza A, et al. Evidence-based recommendations on conventional systemic treatments in psoriasis: systemic review and expert opinion of a panel of dermatologists. J Eur Acad Dermatol Venereol. 2011;25(2):2–11.PubMedCrossRefGoogle Scholar
  48. 48.
    Gottlieb A, Korman NJ, Gordon KB, et al. Guidelines of care for the management of psoriasis and psoriatic arthritis: section 4: guidelines of care for the management and treatment of psoriasis with traditional systemic agents. J Am Acad Dermatol. 2008;58:851–64.PubMedCrossRefGoogle Scholar
  49. 49.
    Esposito M, Mazzotta A, Casciello C, Chimenti S. Etanercept at different dosages in the treatment of generalized pustular psoriasis: a case series. Dermatology. 2008;216(4):355–60.PubMedCrossRefGoogle Scholar
  50. 50.
    Zangrilli A, Papoutsaki M, Talamonti M, Chimenti S. Long-term efficacy of adalimumab in generalized pustular psoriasis. J Dermatolog Treat. 2008;19(3):185–7.PubMedCrossRefGoogle Scholar
  51. 51.
    Alvarez AC, Rodriguez-Nevado I, de Argila D. Recalcitrant pustular psoriasis successfully treated with adalimumab. Pediatr Dermatol. 2011;28(2):195–7.PubMedCrossRefGoogle Scholar
  52. 52.
    Chandran NS, Chong WS. A dramatic response to a single dose of infliximab as rescue therapy in acute generalized pustular psoriasis of von Zumbusch associated with a neutrophilic cholangitis. Australas J Dermatol. 2010;51(1):29–31.PubMedCrossRefGoogle Scholar
  53. 53.
    Torii H, Nakagawa H. Long-term study of infliximab in Japanese patients with plaque psoriasis, psoriatic arthritis, pustular psoriasis and psoriatic erythroderma. J Dermatol. 2011;38(4):321–34.PubMedCrossRefGoogle Scholar
  54. 54.
    Visual Dx—pustulosis, palmoplantar. Visual Dx website. Accessed 29 Sept 2011.
  55. 55.
    Thielen AM, Barde C, Marazza G, Saurat JH. Long-term control with etanercept (Enbrel) of a severe acrodermatitis continua of hallopeau refractory to infliximab (Remicade). Dermatology. 2008;217(2):137–9.PubMedCrossRefGoogle Scholar
  56. 56.
    Lee CS, Koo J. A review of acitretin, a systemic retinoid for the treatment of psoriasis. Expert Opin Pharmacother. 2005;6(10):1725–34.PubMedCrossRefGoogle Scholar
  57. 57.
    Di Lernia V, Guareschi E. Successful treatment of hand and foot psoriasis with infliximab. Dermatol Online J. 2010;16(7):8.PubMedGoogle Scholar
  58. 58.
    Adişen E, Tekin O, Gülekon A, Gürer MA. A retrospective analysis of treatment responses of palmoplantar psoriasis in 114 patients. J Eur Acad Dermatol. 2009;23(7):814–9.CrossRefGoogle Scholar
  59. 59.
    Hofer A, Fink-Puches R, Kerl H, Quehenberger F, Wolf P. Paired comparison of bathwater versus oral delivery of 8-methoxypsoralen in psoralen plus ultraviolet: a therapy for chronic palmoplantar psoriasis. Photodermatol Photoimmunol Photomed. 2006;22(1):1–5.PubMedCrossRefGoogle Scholar
  60. 60.
    Sbidian E, Maza A, Montaudie H, et al. Efficacy and safety of oral retinoids in different psoriasis subtypes: a systematic literature review. J Eur Acad Dermatol Venereol. 2011;25(2):28–33.PubMedCrossRefGoogle Scholar
  61. 61.
    Behrens S, von Kobyletzki G, Gruss C, Reuther T, Altmeyer P, Kerscher M. Puva-bath photochemotherapy (PUVA-soak therapy) of recalcitrant dermatoses of the palms and soles. Photodermatol Photoimmunol Photomed. 1999;15(2):47–51.PubMedCrossRefGoogle Scholar
  62. 62.
    Ettler K, Richards B. Acitretin therapy for palmoplantar pustulosis combined with UVA and topical 8-MOP. Int J Dermatol. 2001;40:541–2.PubMedCrossRefGoogle Scholar
  63. 63.
    Lauharanta J, Geiger JM. A double-blind comparison of acitretin and etretinate in combination with bath PUVA in the treatment of extensive psoriasis. Br J Dermatol. 1989;121:107–12.PubMedCrossRefGoogle Scholar
  64. 64.
    Yawalkar N, Hunger RE. Succesful treatment of recalcitrant palmoplantar pustular psoriasis with sequential use of infliximab and adalimumab. Dermatology. 2009;218(1):79–83.PubMedCrossRefGoogle Scholar
  65. 65.
    Honigsmann H, Schwartz T. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. New York, NY: Mosby [Elsevier]; 2003. p. 2109–25.Google Scholar
  66. 66.
    Lebwohl MG. The skin and systemic disease: a color atlas and text. 2nd ed. New York, NY: Churchill Livingstone; 2004.Google Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of DermatologyMt. Sinai School of MedicineNew YorkUSA
  2. 2.Department of DermatologyMt. Sinai School of MedicineNew YorkUSA

Personalised recommendations