Premium
X‐linked ichthyosis: relation between cholesterol sulphate, dehydroepiandrosterone sulphate and patient's age
Author(s) -
Mario Delfino,
Enrico Maria Procaccini,
G. Illiano,
A. Milone
Publication year - 1998
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1998.02180.x
Subject(s) - ichthyosis , medicine , endocrinology , dehydroepiandrosterone sulfate , dehydroepiandrosterone , cholesterol , steroid , steroid sulfatase , chemistry , androgen , hormone , dermatology
Steroid sulphatase deficiency is a feature of recessive X‐linked ichthyosis (RXLI) that causes the accumulation of sulphated steroids (SS) in various organs and cells. In a previous study, we detected elevated cholesterol sulphate (CS) and dehydroepiandrosterone sulphate (DHEAS) serum levels in a group of 15 RXLI patients selected in a narrow age range. In the present study both CS and DHEAS serum levels were qualitatively and quantitatively determined using gas‐chromatographic analysis in a group of 33 RXLI patients ranging in age from 3 to 70 years. The levels of CS and DHEAS were significantly increased in all patients. Variations in SS were related both to patients' ages and clinical course of the disease. Serum SS levels start to increase in early infancy, peak at puberty, remain elevated in adults and decrease slightly in the elderly.