Ichthyosis Linearis Circumflexa

ABSTRACT

The authors present a 3-year-old boy showing generalized migratory erythema with doubleedged scaling broders since 3 month after birth, Histologic examination revealed an acanthotic epidermis with hyperkeratosis, focal parakeratosis and papillomatosis. The PAS-positive, diastase-resistant amorphous materials were found in th space of parakeratoric scales and cytoplasms of spinous cells just below the parakeraroric lesion. On clectron microscopy, we could observe the round dense bodise in the upper spinous cells and a number of lipoid granules in the horny layer. Our patient showed characteristic clinical, clinical, microscopic and ultrastructural gearures of ichthyosis linearis citcumflexa(ILC). However, he showed no hair shaft abnormalities, which is the common finding of Metherthon/sysdrome(NS) along with ILC and atopic deathesis.