Abstract
The major hematologic problem encountered in epidermolysis bullosa (EB) is anemia. It is multifactorial in origin, and its severity varies with the type of EB. In some cases, the anemia associated with EB can be refractory to therapy. Other hematologic disorders reported in EB include coagulation abnormalities in a small number of patients, and immunological alterations.
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References
Buchbinder L, Lucky AW, Ballard E, et al. Severe infantile epidermolysis bullosa simplex, Dowling-Meara type. Arch Dermatol. 1986; 122:190–198.
Hruby MA, Esterly NB. Anemia in epidermolysis bullosa letalis. Am J Dis Child 1973;125:696–699.
Fine J-D, Tamura T, Johnson L. Blood vitamin and trace metal levels in epidermolysis bullosa. Arch Dermatol 1989; 125:374–379.
Marsden RA, Gowar FJS, MacDonald AF, Main RA. Epidermolysis bullosa of the esophagus with esophageal web formation. Thorax. 1974;29:287–295.
Auerbach M, Witt D, Toler W, Fierstein M, Lerner RG, Ballard H. Clinical use of the total dose intravenous infusion of iron dextran. J Lab Clin Med. 1988; 111: 566–570.
Eschbach JW, Egrie JC, Downing MR, Browne JK, Adamson JW Correction of the anemia of end-stage renal disease with recombinant human erythropoietin: results of a combined phase I and II clinical trial. N Engl J Med. 1987; 316:73–78.
Hochberg MC, Arnold CM, Hogans BB, Spivak JL. Serum immunoreactive erythropoietin in rheumatoid arthritis: impaired response to anemia. Arthritis Rheum. 1988;31:1318–1321.
Cartwright GE. The anemia of chronic disorders. Semin Hematol. 1966; 3:351–375.
Freireich EJ, Miller A, Emerson CP, Ross JF. The effect of inflammation on the utilization of erythrocyte and transferrin bound radioiron for red cell production. Blood. 1957;12:972–983.
Finch CA, Deubelbeiss K, Cook JD, et al. Ferrokinetics in man. Medicine. 1970; 49:17–53.
Tio TH. Blood coagulation and genetic observations in epidermolysis bullosa hereditaria (EBH). Pediatr Indonesia. 1965;5:499–508.
Tio TH, Waardenburg PJ, Vermeulen HJ. Blood coagulation in epidermolysis bullosa hereditaria. Arch Dermatol. 1963;88:24–31.
Goltz RW, Good RA. Benign hyperglobulinemia purpura: relation to Mikulicz’s disese, sicca syndrome, and epidermolysis bullosa dystrophica. Arch Dermatol. 1961;83:26–39.
Gedde-Dahl Jr T, Niewiarowska M, Sotrmorken H. Parameters of hemostasis in epidermolysis bullosa: absence of significant deviations from normal. Acta Derm Venereol (Stockh). 1966; 46:436–442.
Schnyder UW, Jung EG, Salamon T. Zur Klassifizierung, Histogenetik, Gerinnungsphysiologie und Therapie der hereditaren Epidermolysen. Arch Klin Exp Derm. 1964; 220:38.
Fischer T, Lodin A. Biochemical studies in epidermolysis bullosa. Acta Derm Venereol (Stockh). 1966; 46:324–337.
Blomback B, Carlson LA, Franzen S, Zetterqvist E. Turnover of 131-I-labelled fibrinogen in man: studies in normal subjects, in congenital coagulation factor deficiency states, in liver cirrhosis, in polycythemia vera and in epidermolysis bullosa. Acta Med Scand. 1966; 179:557–574.
Law DK, Dudrick SJ, Abdou NI. Immunocompetence of patients with proteincalorie malnutrition: the effects of nutritional repletion. Ann Intern Med. 1973; 79:545–550
Cunningham-Rundles S, Bockman RS, Lin AN, Giardina PV, Hilgartner MW. Physiological and pharmacological effects of zinc on immune response. Ann NY Acad. Sci. 1990;587:113–122.
Gruskay DM. Nutritional management in the child with epidermolysis bullosa. Arch Dermatol. 1988;124:760–761.
Weismann K. Dystrophic epidermolysis bullosa treated umsuccesfully with oral zinc (letter). Arch Dermatol Res. 1985;277:404–405.
Auer IO, Ziemer E, Sommer H. Immune status in Crohn’s disease: V. Decreased in vitro natural killer cell activity in peripheral blood. Clin Exp Immunol. 1980; 42:41–49.
Tyring SK, Chopra V, Johnson L, Fine J-D. Natural killer cell activity is reduced in patients with severe forms of inherited epidermolysis bullosa. Arch Dermatol. 1989;125:797–800.
Chopra V, Tyring SK, Johnson L, Fine J-D. Peripheral blood mononuclear cell subsets in patients with severe inherited forms of epidermolysis bullosa. Arch Dermatol. 1992;128:201–209.
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© 1992 Springer-Verlag New York, Inc.
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Giardina, P.J., Lin, A.N. (1992). Hematologic Problems in Epidermolysis Bullosa. In: Lin, A.N., Carter, D.M. (eds) Epidermolysis Bullosa. Springer, New York, NY. https://doi.org/10.1007/978-1-4612-2914-8_13
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DOI: https://doi.org/10.1007/978-1-4612-2914-8_13
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4612-7717-0
Online ISBN: 978-1-4612-2914-8
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