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Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis

Author
Robert E Kalb, MD
Section Editor
Kristina Callis Duffin, MD
Deputy Editor
Abena O Ofori, MD

INTRODUCTION

Psoriasis is a common skin disorder characterized by the development of erythematous, scaling plaques on the skin and a wide spectrum of clinical presentations. The most common presentation of psoriasis is chronic plaque psoriasis, which manifests as well-defined inflammatory, red, scaling plaques on the skin. Pustular psoriasis is a less common subtype of psoriasis that presents as an acute, subacute, or chronic pustular eruption. Pustular psoriasis primarily affects adults, but can also occur in children.

Pustular psoriasis develops independently or in association with preexisting psoriasis and may occur in a generalized or localized distribution. Generalized pustular psoriasis (GPP) presents as an acute or subacute, widely distributed eruption of pustules arising on inflamed, erythematous skin (picture 1A-C). Some cases of generalized pustular psoriasis may actually represent a new genetic autoinflammatory disease based on mutations in the IL-36 receptor antagonist. Localized forms of pustular psoriasis primarily affect the palms, soles, or extremity digits.

The epidemiology, pathogenesis, and clinical manifestations of pustular psoriasis will be reviewed here. Other forms of psoriasis and the management of pustular psoriasis are reviewed separately. (See "Epidemiology, clinical manifestations, and diagnosis of psoriasis" and "Guttate psoriasis" and "Pustular psoriasis: Management".)

CLASSIFICATION

The clinical presentations of pustular psoriasis traditionally have been classified into generalized and localized forms. The major clinical variants of generalized pustular psoriasis (GPP) include acute GPP (also known as generalized pustular psoriasis of von Zumbusch) and generalized annular pustular psoriasis (also known as subacute GPP) [1]. Acute GPP is characterized by the abrupt onset of numerous pustules, widespread erythema, and systemic symptoms. The terms "pustular psoriasis of pregnancy" and "impetigo herpetiformis" are used to refer to acute GPP that occurs during pregnancy. Generalized annular pustular psoriasis is a less acute presentation of GPP in which patients develop widespread annular or figurate erythematous plaques studded by pustules. (See "Dermatoses of pregnancy", section on 'Pustular psoriasis of pregnancy'.)

Descriptions of localized forms of pustular psoriasis include acrodermatitis continua of Hallopeau, a chronic pustular condition that primarily involves the digits, and palmoplantar pustulosis, a chronic condition in which the pustular eruption is primarily limited to the palms and soles. It is controversial whether palmoplantar pustulosis is a variant of psoriasis or an independent entity [2,3]. Alternative terms used to refer to palmoplantar pustulosis include palmoplantar pustular psoriasis, pustulosis palmoplantaris, and pustulosis palmaris et plantaris. (See 'Localized pustular psoriasis' below and "Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis".)

                  

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Literature review current through: Nov 2016. | This topic last updated: Wed Jan 27 00:00:00 GMT+00:00 2016.
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References
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  1. Choon SE, Lai NM, Mohammad NA, et al. Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia. Int J Dermatol 2014; 53:676.
  2. Brunasso AM, Puntoni M, Aberer W, et al. Clinical and epidemiological comparison of patients affected by palmoplantar plaque psoriasis and palmoplantar pustulosis: a case series study. Br J Dermatol 2013; 168:1243.
  3. Griffiths CE, Christophers E, Barker JN, et al. A classification of psoriasis vulgaris according to phenotype. Br J Dermatol 2007; 156:258.
  4. Van de Kerkhof PC, Nestle FO. Psoriasis. In: Dermatology, 3rd ed, Bolognia JL, Jorizzo JL, Schaffer, JV. (Eds), Elsevier Limited, 2012. Vol 1, p.135.
  5. Parisi R, Symmons DP, Griffiths CE, et al. Global epidemiology of psoriasis: a systematic review of incidence and prevalence. J Invest Dermatol 2013; 133:377.
  6. Zelickson BD, Muller SA. Generalized pustular psoriasis. A review of 63 cases. Arch Dermatol 1991; 127:1339.
  7. Kardaun SH, Kuiper H, Fidler V, Jonkman MF. The histopathological spectrum of acute generalized exanthematous pustulosis (AGEP) and its differentiation from generalized pustular psoriasis. J Cutan Pathol 2010; 37:1220.
  8. Borges-Costa J, Silva R, Gonçalves L, et al. Clinical and laboratory features in acute generalized pustular psoriasis: a retrospective study of 34 patients. Am J Clin Dermatol 2011; 12:271.
  9. Augey F, Renaudier P, Nicolas JF. Generalized pustular psoriasis (Zumbusch): a French epidemiological survey. Eur J Dermatol 2006; 16:669.
  10. de Oliveira ST, Maragno L, Arnone M, et al. Generalized pustular psoriasis in childhood. Pediatr Dermatol 2010; 27:349.
  11. Posso-De Los Rios CJ, Pope E. New insights into pustular dermatoses in pediatric patients. J Am Acad Dermatol 2014; 70:767.
  12. Liao PB, Rubinson R, Howard R, et al. 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.
  13. Ohkawara A, Yasuda H, Kobayashi H, et al. Generalized pustular psoriasis in Japan: two distinct groups formed by differences in symptoms and genetic background. Acta Derm Venereol 1996; 76:68.
  14. Sugiura K, Takemoto A, Yamaguchi M, et al. The majority of generalized pustular psoriasis without psoriasis vulgaris is caused by deficiency of interleukin-36 receptor antagonist. J Invest Dermatol 2013; 133:2514.
  15. Marrakchi S, Guigue P, Renshaw BR, et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011; 365:620.
  16. Li M, Han J, Lu Z, et al. Prevalent and rare mutations in IL-36RN gene in Chinese patients with generalized pustular psoriasis and psoriasis vulgaris. J Invest Dermatol 2013; 133:2637.
  17. Kanazawa N, Nakamura T, Mikita N, Furukawa F. Novel IL36RN mutation in a Japanese case of early onset generalized pustular psoriasis. J Dermatol 2013; 40:749.
  18. Setta-Kaffetzi N, Navarini AA, Patel VM, et al. Rare pathogenic variants in IL36RN underlie a spectrum of psoriasis-associated pustular phenotypes. J Invest Dermatol 2013; 133:1366.
  19. Hayashi M, Nakayama T, Hirota T, et al. Novel IL36RN gene mutation revealed by analysis of 8 Japanese patients with generalized pustular psoriasis. J Dermatol Sci 2014; 76:267.
  20. Körber A, Mössner R, Renner R, et al. Mutations in IL36RN in patients with generalized pustular psoriasis. J Invest Dermatol 2013; 133:2634.
  21. Capon F. IL36RN mutations in generalized pustular psoriasis: just the tip of the iceberg? J Invest Dermatol 2013; 133:2503.
  22. Hüffmeier U, Wätzold M, Mohr J, et al. Successful therapy with anakinra in a patient with generalized pustular psoriasis carrying IL36RN mutations. Br J Dermatol 2014; 170:202.
  23. Rossi-Semerano L, Piram M, Chiaverini C, et al. First clinical description of an infant with interleukin-36-receptor antagonist deficiency successfully treated with anakinra. Pediatrics 2013; 132:e1043.
  24. Sugiura K, Shoda Y, Akiyama M. Generalized pustular psoriasis triggered by amoxicillin in monozygotic twins with compound heterozygous IL36RN mutations: comment on the article by Navarini et al. J Invest Dermatol 2014; 134:578.
  25. Abbas O, Itani S, Ghosn S, et al. Acrodermatitis continua of Hallopeau is a clinical phenotype of DITRA: evidence that it is a variant of pustular psoriasis. Dermatology 2013; 226:28.
  26. Navarini AA, Valeyrie-Allanore L, Setta-Kaffetzi N, et al. Rare variations in IL36RN in severe adverse drug reactions manifesting as acute generalized exanthematous pustulosis. J Invest Dermatol 2013; 133:1904.
  27. Navarini AA, Simpson MA, Borradori L, et al. Homozygous missense mutation in IL36RN in generalized pustular dermatosis with intraoral involvement compatible with both AGEP and generalized pustular psoriasis. JAMA Dermatol 2015; 151:452.
  28. Ellingford JM, Black GC, Clayton TH, et al. A novel mutation in IL36RN underpins childhood pustular dermatosis. J Eur Acad Dermatol Venereol 2016; 30:302.
  29. Jordan CT, Cao L, Roberson ED, et al. Rare and common variants in CARD14, encoding an epidermal regulator of NF-kappaB, in psoriasis. Am J Hum Genet 2012; 90:796.
  30. Ammar M, Jordan CT, Cao L, et al. CARD14 alterations in Tunisian patients with psoriasis and further characterization in European cohorts. Br J Dermatol 2016; 174:330.
  31. Elston GE, Charles-Holmes R, Carr RA. Precipitation of generalized pustular psoriasis by prednisolone. Clin Exp Dermatol 2006; 31:133.
  32. Georgala S, Koumantaki E, Rallis E, Papadavid E. Generalized pustular psoriasis developing during withdrawal of short-term cyclosporin therapy. Br J Dermatol 2000; 142:1057.
  33. Hong SB, Kim NI. Generalized pustular psoriasis following withdrawal of short-term cyclosporin therapy for psoriatic arthritis. J Eur Acad Dermatol Venereol 2005; 19:522.
  34. Wenk KS, Claros JM, Ehrlich A. Flare of pustular psoriasis after initiating ustekinumab therapy. J Dermatolog Treat 2012; 23:212.
  35. Gregoriou S, Kazakos C, Christofidou E, et al. Pustular psoriasis development after initial ustekinumab administration in chronic plaque psoriasis. Eur J Dermatol 2011; 21:104.
  36. 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:e179.
  37. Morales-Múnera C, Vilarrasa E, Puig L. Efficacy of ustekinumab in refractory palmoplantar pustular psoriasis. Br J Dermatol 2013; 168:820.
  38. Daudén E, Santiago-et-Sánchez-Mateos D, Sotomayor-López E, García-Díez A. Ustekinumab: effective in a patient with severe recalcitrant generalized pustular psoriasis. Br J Dermatol 2010; 163:1346.
  39. Andrulonis R, Ferris LK. Treatment of severe psoriasis with ustekinumab during pregnancy. J Drugs Dermatol 2012; 11:1240.
  40. Duckworth L, Maheshwari MB, Thomson MA. A diagnostic challenge: acute generalized exanthematous pustulosis or pustular psoriasis due to terbinafine. Clin Exp Dermatol 2012; 37:24.
  41. Wu MC, Lee JY. Generalized flare of pustular psoriasis induced by PEGylated interferon-α2b therapy for chronic hepatitis C. Australas J Dermatol 2012; 53:e69.
  42. Wee JS, Natkunarajah J, Moosa Y, Marsden RA. Erythrodermic pustular psoriasis triggered by intravesical bacillus Calmette-Guérin immunotherapy. Clin Exp Dermatol 2012; 37:455.
  43. Juanqin G, Zhiqiang C, Zijia H. Evaluation of the effectiveness of childhood generalized pustular psoriasis treatment in 30 cases. Pediatr Dermatol 1998; 15:144.
  44. Navarini AA, Valeyrie-Allanore L, Setta-Kaffetzi N, et al. Generalized pustular eruptions: time to adapt the disease taxonomy to the genetic architecture? J Invest Dermatol 2014; 134:580.
  45. Oumeish OY, Parish JL. Impetigo herpetiformis. Clin Dermatol 2006; 24:101.
  46. 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 Suppl 1:S43.
  47. Varman KM, Namias N, Schulman CI, Pizano LR. Acute generalized pustular psoriasis, von Zumbusch type, treated in the burn unit. A review of clinical features and new therapeutics. Burns 2014; 40:e35.
  48. 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.
  49. Li SP, Tang WY, Lam WY, Wong SN. Renal failure and cholestatic jaundice as unusual complications of childhood pustular psoriasis. Br J Dermatol 2000; 143:1292.
  50. Weedon D. The psoriasiform reaction pattern. In: Weedon's Skin Pathology, 3rd ed, Weedon D, Strutton G, Rubin AI (Eds), Elsevier Limited, Edinburgh 2010. p.71.
  51. Nakai N, Sugiura K, Akiyama M, Katoh N. Acute generalized exanthematous pustulosis caused by dihydrocodeine phosphate in a patient with psoriasis vulgaris and a heterozygous IL36RN mutation. JAMA Dermatol 2015; 151:311.
  52. Eyler JT, Squires S, Fraga GR, et al. Two cases of acute generalized exanthematous pustulosis related to oral terbinafine and an analysis of the clinical reaction pattern. Dermatol Online J 2012; 18:5.
  53. Walsh S, Creamer D. A diagnostic challenge: acute generalized exanthematous pustulosis or pustular psoriasis due to terbinafine: comment. Clin Exp Dermatol 2012; 37:919.
  54. Cheng S, Edmonds E, Ben-Gashir M, Yu RC. Subcorneal pustular dermatosis: 50 years on. Clin Exp Dermatol 2008; 33:229.
  55. Gurgen J, Dorton D. Unusual case of pemphigus vulgaris mimicking localized pustular psoriasis of the hands and feet. Cutis 2010; 86:138.
  56. Matsuo K, Komai A, Ishii K, et al. Pemphigus foliaceus with prominent neutrophilic pustules. Br J Dermatol 2001; 145:132.
  57. Yang Y, Lin M, Huang SJ, et al. A rare presentation of pemphigus vulgaris as multiple pustules. Indian J Dermatol 2010; 55:293.
  58. Kawakami Y, Oyama N, Sakai E, et al. Childhood tinea incognito caused by Trichophyton mentagrophytes var. interdigitale mimicking pustular psoriasis. Pediatr Dermatol 2011; 28:738.
  59. Feily A, Namazi MR, Seifmanesh H. Generalized pustular psoriasis-like dermatophytosis due to Trichophyton rubrum. Acta Dermatovenerol Croat 2011; 19:209.
  60. Sehgal VN, Verma P, Sharma S, et al. Acrodermatitis continua of Hallopeau: evolution of treatment options. Int J Dermatol 2011; 50:1195.
  61. Naldi L, Gambini D. The clinical spectrum of psoriasis. Clin Dermatol 2007; 25:510.
  62. Ranugha PS, Kumari R, Thappa DM. Acrodermatitis continua of hallopeau evolving into generalised pustular psoriasis. Indian J Dermatol 2013; 58:161.