A case of woolly hair nevus associated with pigmentary demarcation lines and heterochromia iridis: Coincidence or a new association?

Abstract

We report a case of woolly hair nevus with pigmentary demarcation lines and heterochromia iridis. Woolly hair nevus is a rare abnormality of the scalp hair characterized by the patch of hair, which is curlier and light colored than the rest of the scalp hair. Association of woolly hair nevus with some other ectodermal defects effecting skin and eyes has been reported before. Here, woolly hair nevus associated with demarcation lines and heterochromia iridis, to our knowledge, have not been previously reported.

A case of progressive evolution of multiple woolly hair nevi in a child

“…Woolly hair nevus may be associated with linear epidermal nevus, nevus comedonicus, café-au-lait spots, hemangiomas, multiple nevi, palmoplantar keratoderma, precocious puberty, ocular defects such as persistent pupillary membrane, loss of retinal pigment epithelium, heterochromia iridis, and cardiac, neurologic, auditory, dental, or skeletal defects. [ 4 ]…”

Woolly hair nevus: case report and review of literature

“…There have been several associations between woolly hair nevus and ophthalmologic (heterochromia iridis, persistent papillary membrane), [16,17], auditory (auditory disturbance), [18], renal, cutaneous (pigmentary demarcation lines, linear epidermal nevus, white sponge nevus), [2,13,16,19], dental (diastema, decayed lower canine teeth), [5,13], and skeletal (growth impairment, brachyphalangy), [18] manifestations. In half the cases, patients with WHN have an ipsilateral verrucous epidermal nevus.…”

Coexistence of pigmentary demarcation line type B and congenital vitiligo: A unique case report and exploration of potential association

“…While the association of PDLs with other dermatological conditions is uncommon, anecdotal reports have described links between PDLs and heterochromia iridis, woolly hair, and periorbital melanosis (Table 1). 1,2 Notably, there has been no documented association between congenital vitiligo and PDLs in the existing literature.…”