BACKGROUND: Heterozygous mutations in CARD14 have been shown to be associated with psoriasis and familial pityriasis rubra pilaris (PRP). Many patients with CARD14 mutations display features of both disorders, which can result in diagnostic uncertainty. In addition, these eruptions are often recalcitrant to conventional psoriasis therapies such as methotrexate, oral retinoids and TNF-alpha inhibitors. OBJECTIVE: We sought to describe the clinical characteristics, family history, and response to therapy in subjects with papulosquamous eruptions due to mutations in CARD14. METHODS: Subjects were referred for genetic testing as part of a registry of patients with inherited disorders of keratinization. DNA was isolated from blood or saliva, and multiplex targeted next generation sequencing or whole exome sequencing was performed. Clinical histories of subjects with CARD14 mutations were reviewed. RESULTS: We identified 15 kindreds with CARD14-associated papulosquamous eruption (CAPE). Characteristic features of CAPE include early age of onset, prominent involvement of the cheeks, chin and ears, family history of psoriasis or PRP, minimal response to conventional topical and systemic psoriasis therapies, and improvement with ustekinumab. LIMITATIONS: Relatively small sample size. CONCLUSIONS: Many subjects with CARD14 mutations display characteristics of both psoriasis and PRP. We propose the term CARD14-associated papulosquamous eruption (CAPE) to describe this spectrum of disease. Patients with clinical features suggestive of CAPE should undergo CARD14 sequencing and may benefit from treatment with ustekinumab.