Phakomatosis pigmentokeratotica | |
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Other names | Organoid nevus with sebaceous differentiation, a speckled-lentiginous nevus, and other associated anomalies |
Specialty | Dermatology |
Phakomatosis pigmentokeratotica is a rare neurocutanous condition characterized by the combination of an organoid sebaceous nevus and speckled lentiginous nevus.[1]: 634–5 [2]: 776 It is an unusual variant of epidermal naevus syndrome.[3] It was first described by Happle et al.[4] It is often associated with neurological or skeletal anomalies such as hemiatrophy, dysaesthesia and hyperhidrosis in a segmental pattern, mild mental retardation, seizures, deafness, ptosis and strabismus.[5]