A 5-year-old girl with a patch of kinky hair in the right parietal region, which had been present since the age of 4 months and different colored eyes since birth referred to our clinic []. The underlying scalp was normal. The affected hair appeared smaller in diameter and lighter than the normal hair color and no structural hair abnormalities were detected under the light microscope. She had slightly hyperpigmented linear lesions mostly marked on the anterolateral of the right arm with midline pectoral and abdominal extension []. The right eye was diffusely brown, and the left was green []. No ophthalmologic, cardiac, neurological or skeletal abnormalities were observed. Nails and teeth were normal. The patient was diagnosed with a woolly hair nevus of the right parietal scalp associated pigmented demarcation lines and heterochromia iridis.
A 6-year-old girl born to nonconsanguineous parents presented with abnormal patch of hair since 4 years. Her parents noticed circumscribed patch of curling and coiling of hair along with change in texture over the vertex of scalp. Birth and development history were normal. Family history was negative for such condition.\nOn examination, there was solitary cicrcumscribed patch of size 5 cm × 6 cm over vertex []. Hair over the patch had an altered texture, light in color, thinner, and tightly coiled. The hair in other areas of scalp was normal. Skin over the patch was normal. Linear epidermal nevus was present over the chin extending to the neck there [] was no evidence of palmoplantar keratoderma and precocious puberty. Based on the above findings, we arrived at a diagnosis of wooly hair nevus.\nLight microscopy of hair revealed irregularities of the cuticle and twisting of hair []. Dermoscopy revealed snake crawl appearance and knotting of hair []. Diagnosis of woolly hair nevus was made, and parents were counseled for benign nature of the condition.