X-linked ichthyosis and ichthyosis vulgaris: comparison of their clinical features based on biochemical analysis.
Thirty cases of X-linked ichthyosis (XLI) and 32 cases of ichthyosis vulgaris (IV) were diagnosed by measuring the steroid sulphatase activity of peripheral blood lymphocytes or the electrophoretic mobility of serum LDL or both. The clinical features of the two conditions were then compared. In both diseases 60-66% of patients had a family history of the condition. Ichthyosis was noted at birth or immediately afterwards in 59% of the patients with XLI while it appeared in infancy in 68% of those with IV. Scales were mostly large and brown or dark brown in patients with XLI, while the majority of patients with IV had small brown or light brown scales. The distribution of the ichthyotic lesions differed in the two types of ichthyosis. On the trunk, the abdomen was more severely involved than the back in 63% of the cases with the XLI, whereas the back was more scaly than the abdomen in 44% of those with IV. On the extremities, the extensor surface was more severely affected than the flexor surface in both types. X-linked ichthyosis was characterized by the presence of lesions in the pre-auricular area, which were found in 93% of the cases with XLI, while only 17% of the IV patients had ichthyotic lesions at this site. Involvement of the preauricular area could be an important clinical feature for distinguishing XLI from IV.
Okano
M
M
Department of Dermatology, Osaka University School of Medicine, Japan.
Kitano
Y
Y
Yoshikawa
K
K
Nakamura
T
T
Matsuzawa
Y
Y
Yuasa
T
T
D000293:Adolescent; D000328:Adult; D000368:Aged; D001192:Arylsulfatases / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D007057:Ichthyosis / Q000097:blood / Q000175:diagnosis / Q000235:genetics*; D007223:Infant; D008077:Lipoproteins, LDL / Q000097:blood; D008297:Male; D008875:Middle Aged; D043266:Steryl-Sulfatase; D014960:X Chromosome
D008077:Lipoproteins, LDL; D001192:Arylsulfatases; D043266:Steryl-Sulfatase