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Lichen Amyloidosis in Alagille Syndrome

Lichen Amyloidosis in Alagille Syndrome Abstract To the Editor.— Alagille's syndrome, due to interlobular biliary duct deficiency, associates various cardiovascular, vertebral, ocular anomalies and especially, a typical facies, making the diagnosis possible at first glance, according to Alagille and coworkers.1 There are also cutaneous signs, however, that are rarely described in the literature. Therefore, it seemed worthwhile to report a case herein. Report of a Case.— When we saw this 22-year-old woman for the first time, we immediately noticed the facial aspect as described by Alagille and coworkers1: a rounded forehead, eyes deep in the sockets with subdued hypertelorism, a saddle nose, and a small chin (Fig 1). The patient complained of an unbearable pruritus with insomnia. Indeed, on the back side of her legs and ankles, there are discretely keratotic, multiple papules in streaks (Fig 2).Microscopic examination of the skin biopsy specimen disclosed deposits of an amorphous substance under a hyperkeratotic epidermis References 1. Alagille D, Odievre M, Gautier M, Dommergues JP. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental and sexual development and cardiac murmur . J Pediatr . 1975;86:63-71.Crossref 2. Jambrosic J, From L, Hanna W. Lichen amyloidosus ultrastructure and pathogenesis . Am J Dermatolpathol . 1984;6:151-158.Crossref 3. Ferrando J, Bassas S, Rodes J, Mascaro JM. Displasia arterio hepatica (sindrome de Alagille) con manifestaciones cutaneas . Actas Dermo-sif 1983;74:87-92. 4. Weston CEM, Burton JL. Xanthomas in the Watson-Alagille syndrome . J Am Acad Dermatol 1987;16:1117-1121.Crossref 5. Moreno-Gimenez JC, Del Rio Mapelli L, Camacho-Martinez F. Le syndrome d'Alagille: etude clinique et histopathologique . Ann Dermatol Venereol 1984;111:225-229. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

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References (6)

Publisher
American Medical Association
Copyright
Copyright © 1991 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1991.01680090156030
Publisher site
See Article on Publisher Site

Abstract

Abstract To the Editor.— Alagille's syndrome, due to interlobular biliary duct deficiency, associates various cardiovascular, vertebral, ocular anomalies and especially, a typical facies, making the diagnosis possible at first glance, according to Alagille and coworkers.1 There are also cutaneous signs, however, that are rarely described in the literature. Therefore, it seemed worthwhile to report a case herein. Report of a Case.— When we saw this 22-year-old woman for the first time, we immediately noticed the facial aspect as described by Alagille and coworkers1: a rounded forehead, eyes deep in the sockets with subdued hypertelorism, a saddle nose, and a small chin (Fig 1). The patient complained of an unbearable pruritus with insomnia. Indeed, on the back side of her legs and ankles, there are discretely keratotic, multiple papules in streaks (Fig 2).Microscopic examination of the skin biopsy specimen disclosed deposits of an amorphous substance under a hyperkeratotic epidermis References 1. Alagille D, Odievre M, Gautier M, Dommergues JP. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental and sexual development and cardiac murmur . J Pediatr . 1975;86:63-71.Crossref 2. Jambrosic J, From L, Hanna W. Lichen amyloidosus ultrastructure and pathogenesis . Am J Dermatolpathol . 1984;6:151-158.Crossref 3. Ferrando J, Bassas S, Rodes J, Mascaro JM. Displasia arterio hepatica (sindrome de Alagille) con manifestaciones cutaneas . Actas Dermo-sif 1983;74:87-92. 4. Weston CEM, Burton JL. Xanthomas in the Watson-Alagille syndrome . J Am Acad Dermatol 1987;16:1117-1121.Crossref 5. Moreno-Gimenez JC, Del Rio Mapelli L, Camacho-Martinez F. Le syndrome d'Alagille: etude clinique et histopathologique . Ann Dermatol Venereol 1984;111:225-229.

Journal

Archives of DermatologyAmerican Medical Association

Published: Oct 1, 1991

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