A new variant of autosomal recessive exfoliative ichthyosis

A. Zvulunov, E. Cagnano, L. Kachko, Z. Shorer, K. Elbedour, H. Stevens

Research output: Contribution to journalArticlepeer-review

6 Scopus citations

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.

Original languageEnglish
Pages (from-to)382-387
Number of pages6
JournalPediatric Dermatology
Volume19
Issue number5
DOIs
StatePublished - 1 Sep 2002
Externally publishedYes

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