This toddler began to develop slowly migrating red scaly plaques with a red double-edged border on the trunk, distal extremities, face at 6 months of age. Over the last several months lesions appeared on the scalp. The plaques on the face, scalp, and abdomen were stable for months while new expanding papules continued to develop on the extremities. Her mother had a similar eruption that also first developed at the end of the first year of life. Erythrokeratoderma variabilis is an autosomal dominant disorder characterized by migratory patches of erythema which may be annular or scalloped and last for days to months. In time these plaques may become fixed and hyperkeratotic. In some patients lesions may also be fixed, sharply demarcated, and hyperkeratotic. Both types of lesions may develop in the same patient. The trunk, buttocks, face, and extensor surfaces of the extremities are most commonly involved. Involvement of the scalp has not been reported. Although lesions may be present at birth, they usually develop later during the first year of life, progress during childhood, and stabilize at puberty. Mutations in the gene for connexin 31 have been shown to cause erythrokeratoderma variabilis. The rash may improve without treatment and oral retinoids may result in clearing.
