Acrokeratoelastoidosis and knuckle pads coexisting in a child

Acrokeratoelastoidosis (AKE) is a marginal papular keratoderma that typically presents in childhood and young adulthood. Childhood cases have exhibited autosomal-dominant inheritance. Acrokeratoelastoidosis is distinct from other palmoplantar marginal papular keratodermas because of its characteristic dermal elastorrhexis with an overlying epithelial dell. We report an 11-year-old boy with multiple translucent hyperkeratotic papules in a linear fashion on the bilateral palmoplantar surfaces characteristic of AKE. He also presented with knuckle pads on the proximal and distal interphalangeal joints that rarely have been reported with AKE, suggesting that AKE and atraumatic knuckle pads may coexist.