A Rare Occurrence of Shagreen Patch on the Face of a Pediatric Patient with Tuberous Sclerosis

Abstract

How to Cite this Article: Ravi A, Veerabhadra D, Richa G, Ngangbam S. A Rare Occurrence of Shagreen Patch on the Face of aPediatric Patient with Tuberous Sclerosis. Iranian Journal of Child Neurology 2012;6(1):39-42. Tuberous sclerosis is a neurocutaneous syndrome characterized by a clinical spectrum varying from severe mental retardation and incapacitating seizures to normal intelligence and lack of seizures, often within the same family(1). The younger the patients present with symptoms and signs of TSC (tuberous sclerosis complex), the greater are the likelihood of mental retardation (2). Skin features are very characteristic of the disease and help in the early diagnosis of the disease. Shagreen patch is one of the major diagnostic features of the disease. It is a less common skin lesion consisting of an excess amount of fibrous tissue similar to that found in scar tissues (3). It is a section of thickened, elevated pebbly skin like an orange peel. The name is derived from the French phrase “peau chagrinee”. It is usually found on the lower back, buttock, thigh and the nape of the neck commonly. In this case report we report a characteristic skin lesion, the shagreen patch in a six-year-old child with TSC at an unusual site (the cheek) never reported before with the best of our knowledge.