Extensive linear epidermal nevus associated with hemangiomas of bones and vitamin-D-resistant rickets

N Stosiek, OP Hornstein, D Hiller, KP Peters - Dermatology, 1994 - karger.com
N Stosiek, OP Hornstein, D Hiller, KP Peters
Dermatology, 1994karger.com
The association of connate, left-sided, extensive epidermal verrucous nevus, multiple
isolated bone tumors and vitamin-D-resistant rickets since childhood seen in a 20-year-old
male patient corresponded to an epidermal nevus syndrome (ENS). However, other organ
involvement occasionally associated with ENS could not be found in this patient, and his
intraosseous tumors represented histologically benign hemangiomas. Serum analysis
revealed hypophosphatemia (together with phosphaturia), decreased levels of 1, 25 …
Abstract
The association of connate, left-sided, extensive epidermal verrucous nevus, multiple isolated bone tumors and vitamin-D-resistant rickets since childhood seen in a 20-year-old male patient corresponded to an epidermal nevus syndrome (ENS). However, other organ involvement occasionally associated with ENS could not be found in this patient, and his intraosseous tumors represented histologically benign hemangiomas. Serum analysis revealed hypophosphatemia (together with phosphaturia), decreased levels of 1,25-dihydroxycholecalciferol and elevated levels of alkaline phosphatase indicating hypophosphatemic osteomalacia. Therefore we suppose that vitamin-D-resistant rickets combined with skeletal tumors represents a peculiar type of osteomalacia caused by unilateral mesenchymomas.
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