Background: Pathogenic mutations in caspase recruitment domain-containing protein 14 (CARD14) lead to CARD14-associated papulosquamous eruption, which shares clinicopathologic findings with psoriasis and pityriasis rubra pilaris. We aimed to describe distinguishing histopathologic features of CARD14-associated papulosquamous eruption.

Methods: This retrospective study examined the histopathologic features of specimens from patients with confirmed CARD14-associated papulosquamous eruption and adult patients with plaque psoriasis and pityriasis rubra pilaris.

Results: Lesional skin biopsies from patients with CARD14-associated papulosquamous eruption consistently showed alternating checkerboard parakeratosis and orthokeratosis, acanthosis without acantholysis, and dilated vessels in the dermal papillae, with some cases also showing follicular plugging.

Conclusion: CARD14-associated papulosquamous eruption has a range of findings, with a predominance of features typically associated with pityriasis rubra pilaris.

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http://dx.doi.org/10.1111/cup.13633DOI Listing

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Article Synopsis
  • Mutations in the caspase activation and recruitment domain are linked to several skin conditions, including psoriasis and atopic dermatitis.
  • A pediatric patient was identified with a new mutation (c.394A > T/-) related to a condition known as CAPE.
  • The patient responded well to treatment with biological therapies.
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A wide range of inherited and acquired conditions can manifest as infantile erythroderma, among which -associated papulosquamous eruption (CAPE) is a rare cause. An infant boy presented with a psoriasiform rash that progressed to erythroderma and was unresponsive to topical steroids and cyclosporine. The early onset of the disease, its severity and resistance to conventional treatment were suggestive of a genetic cause.

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