Dermatology
Case Report
A Case of Congenital Reticular Ichthyosiform Erythroderma – Ichthyosis ‘en confettis’Brusasco A. · Tadini G. · Cambiaghi S. · Ermacora E. · Grimalt R. · Caputo R.Centre for Inherited Cutaneous Diseases, 1st Department of Dermatology and Pediatric Dermatology, IRCCS, Ospedale Maggiore, University of Milan, Italy
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Article / Publication Details
Received: December 11, 1992
Accepted: April 19, 1993
Published online: October 07, 2009
Issue release date: 1994
Number of Print Pages: 6
Number of Figures: 0
Number of Tables: 0
ISSN: 1018-8665 (Print)
eISSN: 1421-9832 (Online)
For additional information: https://www.karger.com/DRM
Abstract
We describe the third case of congenital reticular ichthyosiform erythroderma (CRIE), a rare inherited keratinization disorder, the second with the peculiar reticulate skin pattern. The same case had been previously described and defined, for the clinical appearance, as ichthyosis ‘en confettis’. An 18-year-old girl was born with the clinical features of an erythrodermic lamellar ichthyosis. Patches of normal skin enclosed by erythematous-ichthyotic skin in a reticular arrangement appeared on the trunk at the age of 10 years, and they enlarged slowly during 6 years. The treatment with etretinate, started 2 years ago, further increased this process. Another peculiar clinical feature is a remarkable hypertrichosis. At the ultrastructural level, perinuclear deposits of filamentous material in vacuolized keratinocytes of the upper epidermis, pathognomonic for CRIE, were demonstrated. This suggests that CRIE and ichthyosis ‘en confettis’ are the same disorder. In addition the peculiar clinical presentation of this rare genodermatosis develops only during late childhood and puberty. The identification of three sporadic cases only leaves the problem of inheritance still unsolved.
© 1994 S. Karger AG, Basel
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Article / Publication Details
Received: December 11, 1992
Accepted: April 19, 1993
Published online: October 07, 2009
Issue release date: 1994
Number of Print Pages: 6
Number of Figures: 0
Number of Tables: 0
ISSN: 1018-8665 (Print)
eISSN: 1421-9832 (Online)
For additional information: https://www.karger.com/DRM
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