title: Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia.
abstract: BACKGROUND
  Limited information exists regarding juvenile generalized pustular psoriasis (GPP). We aim to determine the clinical profile and outcome of Malaysians with juvenile GPP.
  
  
  METHODS
  Review of hospital case notes on patients with juvenile GPP.
  
  
  RESULTS
  Twenty-seven patients with juvenile GPP were identified. Female to male ratio was 1.4:1. The median age at onset of GPP was 6.5 years. Ten patients had prior psoriasis with a median pre-pustular duration of 2.7 years. Onset of GPP was earlier in patients without prior psoriasis (5.1 years vs. 12.0 years, P = 0.002). Precipitating factors identified included stress, upper respiratory tract infection, systemic steroid use, vaccination, and pregnancy. A positive family history of psoriasis and GPP was present in six and one patient(s), respectively. Twenty-one patients had acute, five annular, and one localized variant of GPP. Arthritis was present in 22.2%. Fever, leukocytosis, and transaminitis were mainly seen in patients with acute GPP at 80.9, 72.2, and 11.1%, respectively. Among 20 patients screened, eight carry IL36RN variants and one has CARD14 mutation. IL36RN-positive patients have more severe disease characterized by early onset, low prevalence of prior plaque psoriasis, high prevalence of systemic inflammation, and need for continuous long-term systemic therapy. Acitretin and cyclosporine were effective in aborting acute GPP in 100% of 16 and 66.7% of six patients treated, respectively. However, relapses were common. Only three of the 17 patients whose initial acute GPP was controlled with systemic agents were successfully weaned off treatment.
  
  
  CONCLUSIONS
  Juvenile GPP is a chronic recalcitrant disease. IL36RN-positive patients have more severe disease.
authors.affiliation: School of Medicine and Health Sciences, Monash University, Melbourne, Vic., Australia.
authors.forename: Bi-Wen
authors.initials: BW
authors.lastname: Lau
authors.affiliation: School of Medicine and Health Sciences, Monash University, Melbourne, Vic., Australia.
authors.forename: Dee-Zhen
authors.initials: DZ
authors.lastname: Lim
authors.affiliation: Division of Genetics and Molecular Medicine, King's College, London, UK.
authors.forename: Francesca
authors.initials: F
authors.lastname: Capon
authors.affiliation: Division of Genetics and Molecular Medicine, King's College, London, UK.
authors.forename: Jonathan N
authors.initials: JN
authors.lastname: Barker
authors.affiliation: Department of Dermatology Hospital Sultanah Aminah, Johor Bahru, Malaysia.
authors.forename: Siew-Eng
authors.initials: SE
authors.lastname: Choon
mesh_terms: D017255:Acitretin / Q000627:therapeutic use; D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D053468:CARD Signaling Adaptor Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D016572:Cyclosporine / Q000627:therapeutic use; D003879:Dermatologic Agents / Q000627:therapeutic use; D005260:Female; D006162:Guanylate Cyclase / Q000235:genetics; D006801:Humans; D007378:Interleukins / Q000235:genetics; D007641:Keratolytic Agents / Q000627:therapeutic use; D008296:Malaysia / Q000453:epidemiology; D008297:Male; D008565:Membrane Proteins / Q000235:genetics; D011247:Pregnancy; D011248:Pregnancy Complications / Q000209:etiology*; D011565:Psoriasis / Q000188:drug therapy* / Q000209:etiology* / Q000473:pathology; D012141:Respiratory Tract Infections / Q000150:complications; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013256:Steroids / Q000009:adverse effects; D013315:Stress, Psychological / Q000150:complications; D062606:Tertiary Care Centers; D014611:Vaccination / Q000009:adverse effects; D055815:Young Adult
chemical_list: D053468:CARD Signaling Adaptor Proteins; D003879:Dermatologic Agents; C122187:IL36RN protein, human; D007378:Interleukins; D007641:Keratolytic Agents; D008565:Membrane Proteins; D013256:Steroids; D016572:Cyclosporine; C426243:CARD14 protein, human; D006162:Guanylate Cyclase; D017255:Acitretin