Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Epidermolysis Bullosa Hereditaria Letalis

Epidermolysis Bullosa Hereditaria Letalis Abstract To the Editor.— In the report by Pearson et al in the March 1974 issue of the Archives,1 the authors suggested that anemia in patients with epidermolysis bullosa letalis results from bone marrow failure. We recently reported2 extensive hematologic studies performed in two of the patients discussed by Pearson et al.The results of these studies indicated consistently low levels of plasma iron, total iron-binding capacity, and plasma transferrin. Bone marrow examination revealed normal numbers and morphologic characteristics of erythrocyte precursors, as well as absence of stainable iron. Ferrokinetic data included rapid plasma radioactive iron59Fe clearance consistent with iron deficiency and increased plasma and erythrocyte iron turnover rates, indicating a normal to increased rate of erythropoiesis. Erythrocyte (59Fe) utilization at 14 days was slightly reduced, which was consistent with that seen in disease states associated with inflammation or infection, or both. In addition, significantly References 1. Pearson RW, Potter B, Strauss F: Epidermolysis bullosa hereditaria letalis . Arch Dermatol 109:349-355, 1974.Crossref 2. Hruby MA, Esterly NB: Anemia in epidermolysis bullosa letalis . Am J Dis Child 125:696-699, 1973. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Epidermolysis Bullosa Hereditaria Letalis

Archives of Dermatology , Volume 111 (4) – Apr 1, 1975

Loading next page...
 
/lp/american-medical-association/epidermolysis-bullosa-hereditaria-letalis-v3o3DvO3q7

References (4)

Publisher
American Medical Association
Copyright
Copyright © 1975 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1975.01630160117023
Publisher site
See Article on Publisher Site

Abstract

Abstract To the Editor.— In the report by Pearson et al in the March 1974 issue of the Archives,1 the authors suggested that anemia in patients with epidermolysis bullosa letalis results from bone marrow failure. We recently reported2 extensive hematologic studies performed in two of the patients discussed by Pearson et al.The results of these studies indicated consistently low levels of plasma iron, total iron-binding capacity, and plasma transferrin. Bone marrow examination revealed normal numbers and morphologic characteristics of erythrocyte precursors, as well as absence of stainable iron. Ferrokinetic data included rapid plasma radioactive iron59Fe clearance consistent with iron deficiency and increased plasma and erythrocyte iron turnover rates, indicating a normal to increased rate of erythropoiesis. Erythrocyte (59Fe) utilization at 14 days was slightly reduced, which was consistent with that seen in disease states associated with inflammation or infection, or both. In addition, significantly References 1. Pearson RW, Potter B, Strauss F: Epidermolysis bullosa hereditaria letalis . Arch Dermatol 109:349-355, 1974.Crossref 2. Hruby MA, Esterly NB: Anemia in epidermolysis bullosa letalis . Am J Dis Child 125:696-699, 1973.

Journal

Archives of DermatologyAmerican Medical Association

Published: Apr 1, 1975

There are no references for this article.