Multiple Unilateral Zosteriform Connective Tissue Nevi on the Trunk

Connective tissue nevus is not a true tumor, but rather a hamartoma involving various components of connective tissue. It presents as a slow-growing, painless, flesh-colored, or pink nodule or plaque that is evident from childhood. While any region of the body may be affected, there is a predilection for the trunk and extremities. A 20-month-old girl presented with three ipsilateral confluent popular plaques with zosteriform distribution that had formed over the previous 17 months on the left chest and abdomen. The patient remained asymptomatic. Unlike all previously reported cases demonstrating a single lesion, we report a connective tissue nevi in a child who presented with multiple unilateral zosteriform lesions, an unusual pattern of distribution without evidence of tuberous sclerosis complex.