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  • Title: Phacomatosis pigmentokeratotica associated with a suprasellar dermoid cyst and leg hypertrophy.
    Author: Majmudar V, Loffeld A, Happle R, Salim A.
    Journal: Clin Exp Dermatol; 2007 Nov; 32(6):690-2. PubMed ID: 17868392.
    Abstract:
    Phacomatosis pigmentokeratotica (PP) is a mosaic disorder that represents a distinct epidermal naevus syndrome. Its defining features are an epidermal naevus that is usually of the sebaceous type and a speckled lentiginous naevus arranged in a chequerboard pattern. In addition, there are neurological, ophthalmological and skeletal abnormalities, including limb hemiatrophy with muscular weakness, ptosis, seizures and ipsilateral segmental hyperaesthesia and hyperhidrosis. We report a 44-year-old man with an extensive epidermal naevus and an ipsilateral speckled lentiginous naevus. He also had ipsilateral right leg hypertrophy and a suprasellar dermoid cyst with associated neurological abnormalities. We propose that this case represents an unusual example of PP.
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