Previously: Pityriasis rubra pilaris type V
Prominent facial involvement, pink-red coalescing plaques, localized or generalized scaling/erythema. Can have ectropion when severe.
Pink-red coalescing plaques with areas of sparing. Some individuals have pustules within plaques.
Some individuals have joint pain.
Ustekinumab and Th17 inhibitors work well (Takeichi, 2017; Craiglow, 2018).
Takeichi T, Sugiura K, Nomura T et al. Pityriasis rubra pilaris type V as an autoinflammatory disease by CARD14 mutations. JAMA Dermatol 2017; 153:66-70. || Craiglow BG, Boyden LM, Hu R et al. CARD14-associated papulosquamous eruption: a spectrum including features of psoriasis and pityriasis rubra pilaris. J Am Acad Dermatol 2018; 79:487-94.
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