[Skip to Navigation]
April 1975

Epidermolysis Bullosa Hereditaria Letalis

Arch Dermatol. 1975;111(4):527-528. doi:10.1001/archderm.1975.01630160117023

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