|LETTER TO EDITOR
|Year : 1996 | Volume
| Issue : 4 | Page : 269-270
Cutis verticis gyrata with epilepsy
Jeetendra Saraswat, CM Kuldeep
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Saraswat J, Kuldeep C M. Cutis verticis gyrata with epilepsy. Indian J Dermatol Venereol Leprol 1996;62:269-70
| To the Editor,|| |
A 28-year-old man presented with corrugated scalp, abnormal physical growth, poor mental development and epileptic seizures since early childhood. Family and personal history did not suggest genetic and systemic disease.
On physical examination, there were multiple furrows and folds of 2-4 cm depth and 20-30 cm length, which were arranged sagitally over the scalp. Psychometry suggested mental age of 5 years and IQ was 35. EEG showed generalised paroxysmal epileptiform activity. Other routine haematological, biochemical and radiological investigations were normal. He was provided anti-epileptic treatment and suggested for plastic surgery of the scalp.
Though cutis verticis gyrata was coined by Unna in 1907 for cerebriform appearance of the skin, Fisher (1922) provided aetiopathological details of the disorder. Various systemic and cutaneous inflammatory disorders may result into cutis verticis gyrata. Nevertheless autosomal recessive or dominant inheritance may be found in some cases. The male predominance in cutis verticis gyrata may be because of androgenic hormones and a lethal factor operating in female foetuses. A distinct Lennox-Gastaut syndrome has been described to show a symmetrical spike wave discharges at less than 3Hz even in sleep. Rotational traction over scalp hair can produce cutis verticis gyrata in normal person.
The underlying pathology responsible for thickening of the scalp lies in abnormal proliferation of collagen and reticular fibres, deposition of mucopolysaccharides in corium and epithelial hyperplasia. In the index case evidence of mental retardation and the distinct EEG pattern without any other systemic abnormality indicates primary cutis verticis gyrata.
| References|| |
|1.||Polar S, Butterworth T. Cutis verticis gyrata: review with report of seven new cases. Am J Ment Defic 1953;57:613-31. |
|2.||Paulson GW. Cutis verticis gyrata and the lennox syndrome. Develop Med Child Neurol 1974;16:196-200. [PUBMED] |
|3.||Khare AK, Singh G. Acquired cutis verticis gyrata due to rotational traction. Br J Dermatol 1984;110:125-6. [PUBMED] |