Back to Search
Start Over
A New Variant of Autosomal Recessive Exfoliative Ichthyosis.
- Source :
-
Pediatric Dermatology . Sep/Oct2002, Vol. 19 Issue 5, p382. 6p. 2 Color Photographs, 3 Black and White Photographs, 1 Diagram, 1 Chart. - Publication Year :
- 2002
-
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. [ABSTRACT FROM AUTHOR]
- Subjects :
- *ICHTHYOSIS
*GENETIC disorders
Subjects
Details
- Language :
- English
- ISSN :
- 07368046
- Volume :
- 19
- Issue :
- 5
- Database :
- Academic Search Index
- Journal :
- Pediatric Dermatology
- Publication Type :
- Academic Journal
- Accession number :
- 7784336
- Full Text :
- https://doi.org/10.1046/j.1525-1470.2002.00111.x