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

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.

Figure 1

Psoriasiform eruption in a 5 month-old Hispanic female with KD diagnosed and treated with IVIG and aspirin on illness day 13. Patient received infliximab on day 15 for severe coronary artery dilation. This eruption developed on day 22, consisting of (A) well-demarcated pink-red plaques, with (B) extensive crusting. The skin disease was treated with subcutaneous etanercept for 6 months until resolution, with no recurrence in almost 4 years.

Figure 1

Psoriasiform eruption in a 5 month-old Hispanic female with KD diagnosed and treated with IVIG and aspirin on illness day 13. Patient received infliximab on day 15 for severe coronary artery dilation. This eruption developed on day 22, consisting of (A) well-demarcated pink-red plaques, with (B) extensive crusting. The skin disease was treated with subcutaneous etanercept for 6 months until resolution, with no recurrence in almost 4 years.

Figure 2

Psoriasiform eruption in a 3 year-old non-Hispanic White female with KD diagnosed and treated with IVIG and aspirin on illness day 5, followed by infliximab on day 7 due to IVIG nonresponse. Pink plaques with fine white scale (A and B) appeared 2 months later. The eruption was treated with low potency topical steroids, and gradually resolved over 12 months.

Figure 2

Psoriasiform eruption in a 3 year-old non-Hispanic White female with KD diagnosed and treated with IVIG and aspirin on illness day 5, followed by infliximab on day 7 due to IVIG nonresponse. Pink plaques with fine white scale (A and B) appeared 2 months later. The eruption was treated with low potency topical steroids, and gradually resolved over 12 months.

Figure 3

Kaplan-Meier plots of eruption duration. The green line represents thegroup with psoriasiform eruptions during Kawasaki disease. The blue line represents the group of matched controls with conventional psoriasis (not associated with KD).

Figure 4

Histopathologic examination of a KD-associated psoriasiform lesion, demonstrating (A) psoriasiform hyperplasia and focal thinning of the suprapapillary plates (H&E, 40x), with (B) mounds of parakeratosis, neutrophils, and serum in the cornified layer (H&E, 200x). Immunohistochemistry showed (C) suprabasilar keratin 16 staining (100x) and (D) increased Ki-67 antigen expression in the lower epidermis (40x). Non-psoriatic skin typically has keratin 16 staining only in the basal cells and the tips of rete ridges, while Ki-67 expression is scant and at most present at the basal layer.^–,

Figure 4

Histopathologic examination of a KD-associated psoriasiform lesion, demonstrating (A) psoriasiform hyperplasia and focal thinning of the suprapapillary plates (H&E, 40x), with (B) mounds of parakeratosis, neutrophils, and serum in the cornified layer (H&E, 200x). Immunohistochemistry showed (C) suprabasilar keratin 16 staining (100x) and (D) increased Ki-67 antigen expression in the lower epidermis (40x). Non-psoriatic skin typically has keratin 16 staining only in the basal cells and the tips of rete ridges, while Ki-67 expression is scant and at most present at the basal layer.^–,

Figure 4

Histopathologic examination of a KD-associated psoriasiform lesion, demonstrating (A) psoriasiform hyperplasia and focal thinning of the suprapapillary plates (H&E, 40x), with (B) mounds of parakeratosis, neutrophils, and serum in the cornified layer (H&E, 200x). Immunohistochemistry showed (C) suprabasilar keratin 16 staining (100x) and (D) increased Ki-67 antigen expression in the lower epidermis (40x). Non-psoriatic skin typically has keratin 16 staining only in the basal cells and the tips of rete ridges, while Ki-67 expression is scant and at most present at the basal layer.^–,

Figure 4

Histopathologic examination of a KD-associated psoriasiform lesion, demonstrating (A) psoriasiform hyperplasia and focal thinning of the suprapapillary plates (H&E, 40x), with (B) mounds of parakeratosis, neutrophils, and serum in the cornified layer (H&E, 200x). Immunohistochemistry showed (C) suprabasilar keratin 16 staining (100x) and (D) increased Ki-67 antigen expression in the lower epidermis (40x). Non-psoriatic skin typically has keratin 16 staining only in the basal cells and the tips of rete ridges, while Ki-67 expression is scant and at most present at the basal layer.^–,