You are seeing this message because your Web browser does not support basic Web standards. Find out more about why this message is appearing and what you can do to make your experience on this site better.


ABOUT ARCHIVES
Advanced Search

Welcome   | My Account | E-mail Alerts | Access Rights | Sign In


  Vol. 127 No. 2, February 1991 TABLE OF CONTENTS
  Archives
  •  Online Features
  OBSERVATIONS
 This Article
 •References
 •Full text PDF
 •Send to a friend
 • Save in My Folder
 •Save to citation manager
 •Permissions
 Citing Articles
 •Citing articles on HighWire
 •Contact me when this article is cited
 Related Content
 •Similar articles in this journal
 Social Bookmarking
  Add to CiteULike Add to Connotea Add to Del.icio.us Add to Digg Add to Reddit Add to Technorati Add to Twitter What's this?

Ichthyosis, Exocrine Pancreatic Insufficiency, Impaired Neutrophil Chemotaxis, Growth Retardation, and Metaphyseal Dysplasia (Shwachman Syndrome)

Report of a Case With Extensive Skin Lesions (Clinical, Histological, and Ultrastructural Findings)

Marleen Goeteyn; Arnold P. Oranje; Vojislav D. Vuzevski; Ronald de Groot; Lisette W. A. van Suijlekom-Smit

Arch Dermatol. 1991;127(2):225-230.


Abstract

• The Shwachman syndrome comprises exocrine pancreatic insufficiency, growth retardation, and bone marrow hypoplasia resulting in neutropenia. Clinical, morphological, and ultrastructural studies, as well as hair analysis, were performed in a patient with Shwachman's syndrome and severe ichthyosis. Clinical findings were lamellar ichthyosiform desquamation on the extremities. The hair was scanty and short on the scalp, in the eyelashes, and in the eyebrows. The nails were hyperkeratotic. Morphologic findings were slight, regular acanthosis and severe diffuse hyperkeratosis with variable parakeratosis. The granular layer was thickened. The papillary dermis showed very slight perivascular lymphocyte infiltration. The most prominent ultrastructural finding was the presence of solitary or multiple droplets of varying size in the cytoplasm of the keratinocytes. Hair analysis revealed no abnormalities; the cystine concentration in hair specimens was normal.

(Arch Dermatol. 1991;127:225-230)



Author Affiliations

From the Departments of Dermatovenereology (Division of Pediatric Dermatology) (Drs Goeteyn and Oranje), Pathology (Dr Vuzevski), and Pediatrics (Drs de Groot and van Suijlekom-Smit), Erasmus University and University Hospital Rotterdam, Sophia Children's Hospital, Rotterdam, the Netherlands. Dr Goeteyn is presently with the Department of Dermatology, Ghent (Belgium) University Hospital.


Footnotes

Accepted for publication August 31, 1990.

Reprint requests to Department of Dermatovenereology (Division of Pediatric Dermatology), University Hospital Rotterdam, Sophia Children's Hospital, Gordelweg 160, 3038 GE Rotterdam, the Netherlands (Dr Oranje).



Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Ichthyosis, Exocrine Pancreatic Insufficiency, Impaired Neutrophil Chemotaxis, Growth Retardation, and Metaphyseal Dysplasia
Schmidt
Arch Dermatol 1991;127:1417-1418.
ABSTRACT  

Ichthyosis, Exocrine Pancreatic Insufficiency, Impaired Neutrophil Chemotaxis, Growth Retardation, and Metaphyseal Dysplasia-Reply
Oranje et al.
Arch Dermatol 1991;127:1418-1418.
ABSTRACT  





HOME | CURRENT ISSUE | PAST ISSUES | TOPIC COLLECTIONS | CME | SUBMIT | SUBSCRIBE | HELP
CONDITIONS OF USE | PRIVACY POLICY | CONTACT US | SITE MAP
 
© 1991 American Medical Association. All Rights Reserved.