Psoriasiform eruptions during Kawasaki disease (KD): A distinct phenotype

J Am Acad Dermatol. 2016 Jul;75(1):69-76.e2. doi: 10.1016/j.jaad.2016.02.1146. Epub 2016 Mar 4.

Abstract

Background: A psoriasis-like eruption develops in a subset of patients with Kawasaki disease (KD).

Objective: We sought to systematically compare KD-associated psoriasiform eruptions with classic psoriasis and the outcomes of KD in children with and without this rash.

Methods: This was a retrospective study of 11 KD cases with a psoriasiform eruption matched 1:2 by age, gender, and ethnicity with psoriasis-only and KD-only controls. Genotyping was performed in 10 cases for a deletion of 2 late cornified envelope (LCE) genes, LCE3C_LCE3B-del, associated with increased risk for pediatric-onset psoriasis.

Results: Similar to classic psoriasis, KD-associated eruptions were characterized clinically by well-demarcated, scaly pink plaques and histopathologically by intraepidermal neutrophils, suprabasilar keratin 16 expression, and increased Ki-67 expression. They showed less frequent diaper area involvement, more crust and serous exudate, and an enduring remission (91% vs 23% with confirmed resolution; P < .001). Frequency of LCE3C_LCE3B-del and major KD outcomes were similar between cases and controls.

Limitations: The study was limited by the small number of cases, treatment variation, and availability of skin biopsy specimens.

Conclusions: Although the overall clinical and histopathologic findings were similar to conventional psoriasis, this appears to be a distinct phenotype with significantly greater propensity for remission. No adverse effect on KD outcomes was noted.

Keywords: Kawasaki disease; Ki-67; LCE3C_LCE3B deletion; keratin 16; psoriasiform; psoriasis.

MeSH terms

  • Adolescent
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Cornified Envelope Proline-Rich Proteins / genetics
  • Female
  • Genotype
  • Humans
  • Infant
  • Infant, Newborn
  • Keratin-16 / analysis
  • Ki-67 Antigen / analysis
  • Male
  • Mucocutaneous Lymph Node Syndrome / complications*
  • Mucocutaneous Lymph Node Syndrome / genetics
  • Mucocutaneous Lymph Node Syndrome / pathology*
  • Phenotype
  • Prognosis
  • Psoriasis / etiology*
  • Psoriasis / genetics
  • Psoriasis / metabolism
  • Psoriasis / pathology*
  • Retrospective Studies
  • Sequence Deletion

Substances

  • Cornified Envelope Proline-Rich Proteins
  • Keratin-16
  • Ki-67 Antigen
  • LCE3B protein, human
  • LCE3C protein, human