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D. Alagille, M. Odiévre, M. Gautier, Dommergues Jp (1975)
Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur.The Journal of pediatrics, 86 1
Del Rio Mapelli L Moreno-Gimenez JC (1984)
Le syndrome d'Alagille: etude clinique et histopathologiqueAnn Dermatol Venereol, 111
Bassas S Ferrando J (1983)
Displasia arterio hepatica (sindrome de Alagille) con manifestaciones cutaneasActas Dermo-sif, 74
C. Weston, J. Burton (1987)
Xanthomas in the Watson-Aiagille syndromeJournal of The American Academy of Dermatology, 16
J. Giménez, L. Mapelli, F. Martínez (1984)
[Alagille's syndrome. Clinical and histo-pathologic study].Annales De Dermatologie Et De Venereologie, 111
J. Jambrosic, L. From, W. Hanna, D. King, S. Rabson (1984)
Lichen amyloidosus: Ultrastructure and pathogenesisThe American Journal of Dermatopathology, 6
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.
Archives of Dermatology – American Medical Association
Published: Oct 1, 1991
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