title: A new variant of autosomal recessive exfoliative ichthyosis.
abstract: We report unusual congenital ichthyosiform dermatosis in 5 of 12 children in two related families of unaffected, consanguineous Bedouin parents. It appeared shortly after birth as a fine peeling of nonerythematous skin on palms and soles. Gradually it evolved into prominent, well-demarcated areas of peeling skin in moist and traumatized regions. The cutaneous manifestations share features of ichthyosis bullosa of Siemens (IBS) and peeling skin syndrome (PSS). Histologic examination revealed orthokeratosis, a thickened granular cell layer, and spongiosis without epidermolytic hyperkeratosis. On electron microscopy there was prominent intercellular edema and numerous aggregates of keratin filaments in basal keratinocytes. This combination of clinical, histologic, and ultrastructural features has not been previously reported in the heterogeneous group of congenital ichthyoses. We suggest that it represents a new variant of exfoliative ichthyosis.
authors.affiliation: Departments of Dermatology, Soroka University Medical Center and Faculty of Health Sciences, Ben Gurion University, Beer Sheva, Israel. azvulun@bgumail.bgu.ac.il
authors.forename: A
authors.initials: A
authors.lastname: Zvulunov
authors.forename: E
authors.initials: E
authors.lastname: Cagnano
authors.forename: L
authors.initials: L
authors.lastname: Kachko
authors.forename: Z
authors.initials: Z
authors.lastname: Shorer
authors.forename: K
authors.initials: K
authors.lastname: Elbedour
authors.forename: H
authors.initials: H
authors.lastname: Stevens
mesh_terms: D000293:Adolescent; D003879:Dermatologic Agents / Q000627:therapeutic use; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D007057:Ichthyosis / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010375:Pedigree
chemical_list: D003879:Dermatologic Agents