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CARD14-associated papulosquamous eruption
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CARD14-associated papulosquamous eruption

Contributors: Negar Esfandiari BHSc, Keith Morley MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

CARD14-associated papulosquamous eruption (CAPE) is an autosomal dominant inflammatory disorder caused by mutations in the CARD14 (caspase recruitment domain family member 14) gene. The cutaneous findings in CAPE resemble both psoriasis and pityriasis rubra pilaris (PRP). It can be distinguished from these dermatoses as it manifests early in life, often by the age of one year.

There are fewer than 40 cases reported in the literature to date. It is likely that the prevalence of CAPE is higher than stated, given that genetic testing may not have been performed on many patients with treatment refractory to psoriasis and PRP.

CAPE is caused by gain of function mutations in the CARD14 gene, which is expressed in epithelial cells and keratinocytes. The CARD14 gene is involved with activating the nuclear factor kappa B (NF-κB) pathway and increasing downstream inflammatory interleukins and intermediates, such as interleukin (IL)-17, IL-22, and IL-23.

Codes

ICD10CM:
L41.8 – Other parapsoriasis

SNOMEDCT:
22585003 – Papulosquamous dermatosis

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Last Reviewed:01/09/2022
Last Updated:01/24/2022
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CARD14-associated papulosquamous eruption
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A medical illustration showing key findings of CARD14-associated papulosquamous eruption
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