Phakomatosis pigmentokeratotica

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Phakomatosis pigmentokeratotica (pronunciation: fak-oh-ma-toh-sis pig-men-toh-ker-ah-toh-ti-ka) is a rare, complex cutaneous condition characterized by the coexistence of a sebaceous nevus and a speckled lentiginous nevus. The term is derived from the Greek words phakos (meaning birthmark), pigmento (referring to pigmentation), and keratotica (referring to keratin, a protein found in the skin).

Definition

Phakomatosis pigmentokeratotica is a type of epidermal nevus syndrome that is characterized by the presence of both a sebaceous nevus and a speckled lentiginous nevus. It is considered a variant of Schimmelpenning syndrome, which is a group of disorders characterized by skin lesions and abnormalities in other body systems.

Symptoms

The main symptoms of Phakomatosis pigmentokeratotica include the presence of a sebaceous nevus, which is a skin lesion that is present at birth and grows with the individual, and a speckled lentiginous nevus, which is a pigmented skin lesion that appears speckled due to the presence of multiple colors. Other symptoms can include neurological abnormalities, skeletal abnormalities, and ocular abnormalities.

Treatment

Treatment for Phakomatosis pigmentokeratotica is primarily focused on managing the symptoms and can include surgical removal of the skin lesions, laser therapy, and medication to manage any associated neurological symptoms.

See Also

External links

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