Unilateral proteus syndrome in a neonate: A very rare presentation

Abstract

Proteus syndrome (PS) is a rare sporadic disorder with postnatal asymmetric overgrowth from any of the three germinal layers. The tissue overgrowth may present at birth but becomes more conspicuous with the progression of age. Though it apparently can affect any tissue, it commonly involves skin, bones, and central nervous system. The PS is difficult to diagnose as any part of the body is affected along with several complications. To overcome the diagnostic confusion, there are three general mandatory criteria with at least one of the three category signs designated as specific criteria according to First National Conference on PS. We diagnosed our case by connective tissue nevus in sole with three general criteria. Huge cerebriform nevus sebaceous as epidermal nevus was not found previously. A few neonatal PS cases were reported. All the lesions were unilaterally distributed. Our case was neonatal PS with unilateral distribution. We report this case because of its rarity.