Abstract: A 41-year-old male presented with erythematous, desquamative dermatitis  all over the body for 40 days. The clinical manifestation and biopsy features of the lesions were consistence with both pityriasis rubra pilaris and psoriasis, with negative family history. The age of first onset was 41 years old. Preliminary diagnosis was pityriasis rubra pilaris (Type I). Systemic acitretin shows significant improvement. Whole-exome and Sanger DNA sequencing were used to characterize the disease gene in the patient, and a novel heterozygous mutation c.1799 C>T(p.Pro600Leu) in the CARD14 gene was identified. The final diagnosis of CAPE (CARD14-associated papulosquamous eruption) was made according to the clinical and pathological features, as well as the genetic results.

Key words: CARD14, CAPE, pityriasis rubra pilaris, psoriasis