Nevo intradérmico cerebriforme como causa de Cutis verticis gyrata

Abstract

Cerebriform intradermal nevus as a Cause of cutis verticis gyrata 639Rev Assoc Med Bras 2010; 56(6): Cutis verticis gyrata (CVG) is a rare disease that is characterized by excess scalp skin, producing thick folds that form creases and ridges similar in appearance to the whorls of the cerebral cortex. It was first described in the literature by Robert in 1843 and the name cutis verticis gyrata was proposed by Unna in 1907 and remains the accepted name to date. 1,2 It has an estimated prevalence of one case in every 100,000 men and 0,026 cases for every 100,000 women. 3 It can be an isolated manifestation that is present from birth or it can be part of other syndromes.Cutis verticis gyrata is classified as primary (subdivided into essential and non essential) or secondary. The essential primary form is not associated with neurological or ophthalmological disorders, and only the folds on the scalp mimicking the cerebral whorls appear. It appears in puberty and affects more men than women. The non essential primary form affects 0.5% of patients with mental retardation. Cerebral palsy, epilepsy, cataracts and blindness may also be present. 2,4,5,6 The secondary forms of CVG generally occur as a result of inflammatory or neoplastic processes that cause changes to the structure of the scalp. 2,6 One of the rarest forms of secondary CVG is the nevoid form, in which it is melanocytic intradermal nevi that cause the cutaneous hypertrophy. This condition is known as cerebriform intradermal nevus (CIN) and was first linked with CVG in 1937 by Hammond and Ransom. 7 Patients affected by CIN have normal intelligence and are free from other local or systemic diseases.The objective of this paper is to describe a rare case of cerebriform intradermal nevus, discussing its histopathological and clinical features. CaseA white female patient, 43 years old, presented at the Instituto de Dermatologia Professor Azulay with large dimension lesions on the scalp. She stated that at birth she had had a small normal colored macula on her scalp and that it had gradually developed, reaching its largest size during puberty. On physical examination, multiple patches of alopecia were observed in addition to a normal-colored convoluted mass covering the right parietal, right temporal and right occipital regions and small areas of foulsmelling seborrheic dermatitis ( Figures 1A and 1B). Neurological and ophthalmological test results were normal. Family history included no relevant details.X-rays and computerized tomography of the head did not reveal damage to the calvarium. Blood tests and liver, kidney and thyroid function were normal. A cutaneous biopsy revealed melanocytic intradermal nevus with progressive maturation inwards towards the depth of the tissues. Nests and bundles reached the deep dermis and parts of the subcutaneous zone, thereby indicating the intradermal cerebriform subtype (Figures 2 and 3). DisCussionCutis verticis gyrata is a rare disease that is characterized by excess scalp skin, producing folds that are reminiscent of th...