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Dermatomyositis With Cerebral Vasculitis in a Patient With Agammaglobulinemia

Dermatomyositis With Cerebral Vasculitis in a Patient With Agammaglobulinemia Abstract This paper describes a case of dermatomyositis occurring in a patient with agammaglobulinemia. A major manifestation of the disease process was subarachnoid hemorrhage, presumably due to central nervous system vasculitis. Typical changes of dermatomyositis were found in the skin, muscle, gastrointestinal tract, endocardium, epididymis, and brain. No immunoglobulins or β1 C-globulin were localized in the sections of skin or muscle. The failure of the patient's peripheral blood lymphocytes to respond to specific antigens in vitro suggests a qualitative disorder of cell-mediated immunity which may have predisposed to the development of dermatomyositis. References 1. Gitlin D, Janeway CA, Apt L, et al: Agammaglobulinemia, in Lawrence HS (ed): Cellular and Humoral Aspects of the Hypersensitive States . New York, PB Hoeber Inc, 1959, pp 375-441. 2. Good RA, Rotstein J, Mazzitello WF: The simultaneous occurrence of rheumatoid arthritis and agammaglobulinemia . J Lab Clin Med 49:343-357, 1957. 3. Good RA, Gabrielsen AE: Agammaglobulinemia and hypogammaglobulinemia: Relationship to the mesenchymal disease , in Uhr JW (ed): The Streptococcus, Rheumatic Fever and Glomerulonephritis . Baltimore, Williams & Wilkins Co, 1964, pp 368-403. 4. Janeway CA, Gitlin D, Craig JM, et al: " Collagen disease" in patients with congenital agammaglobulinemia . Trans Assoc Amer Physicians 69:93-97, 1956. 5. Blaker F, Fischer K: Dermatomyositis and autoimmune anemia in severe antibody deficiency syndromes . Mschr Kinderheilk 115:453-457, 1966. 6. Rosen FS, Janeway CA: The gamma globulins: III. The antibody deficiency syndromes . New Eng J Med 275:709-715, 1966.Crossref 7. Gotoff SP: Lymphocytes in congenital immunological deficiency diseases . Clin Exp Immunol 3:843-856, 1968. 8. Glaser GH: Collagen diseases and the nervous system . Med Clin N Amer 47:1475-1495, 1963. 9. Mozai T: Involvement of the central nervous system in collagen disease . Jap Circ J 30:1292-1299, 1966. 10. Banker BO, Victor M: Dermatomyositis (systemic angiopathy) of childhood . Medicine 45:261-289, 1966.Crossref 11. Oppenheim JJ: Relationship of in vitro lymphocyte transformation to delayed hypersensitivity in guinea pigs and man . Fed Proc 27:21, 1968. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Dermatomyositis With Cerebral Vasculitis in a Patient With Agammaglobulinemia

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References (14)

Publisher
American Medical Association
Copyright
Copyright © 1972 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1972.02110070103015
Publisher site
See Article on Publisher Site

Abstract

Abstract This paper describes a case of dermatomyositis occurring in a patient with agammaglobulinemia. A major manifestation of the disease process was subarachnoid hemorrhage, presumably due to central nervous system vasculitis. Typical changes of dermatomyositis were found in the skin, muscle, gastrointestinal tract, endocardium, epididymis, and brain. No immunoglobulins or β1 C-globulin were localized in the sections of skin or muscle. The failure of the patient's peripheral blood lymphocytes to respond to specific antigens in vitro suggests a qualitative disorder of cell-mediated immunity which may have predisposed to the development of dermatomyositis. References 1. Gitlin D, Janeway CA, Apt L, et al: Agammaglobulinemia, in Lawrence HS (ed): Cellular and Humoral Aspects of the Hypersensitive States . New York, PB Hoeber Inc, 1959, pp 375-441. 2. Good RA, Rotstein J, Mazzitello WF: The simultaneous occurrence of rheumatoid arthritis and agammaglobulinemia . J Lab Clin Med 49:343-357, 1957. 3. Good RA, Gabrielsen AE: Agammaglobulinemia and hypogammaglobulinemia: Relationship to the mesenchymal disease , in Uhr JW (ed): The Streptococcus, Rheumatic Fever and Glomerulonephritis . Baltimore, Williams & Wilkins Co, 1964, pp 368-403. 4. Janeway CA, Gitlin D, Craig JM, et al: " Collagen disease" in patients with congenital agammaglobulinemia . Trans Assoc Amer Physicians 69:93-97, 1956. 5. Blaker F, Fischer K: Dermatomyositis and autoimmune anemia in severe antibody deficiency syndromes . Mschr Kinderheilk 115:453-457, 1966. 6. Rosen FS, Janeway CA: The gamma globulins: III. The antibody deficiency syndromes . New Eng J Med 275:709-715, 1966.Crossref 7. Gotoff SP: Lymphocytes in congenital immunological deficiency diseases . Clin Exp Immunol 3:843-856, 1968. 8. Glaser GH: Collagen diseases and the nervous system . Med Clin N Amer 47:1475-1495, 1963. 9. Mozai T: Involvement of the central nervous system in collagen disease . Jap Circ J 30:1292-1299, 1966. 10. Banker BO, Victor M: Dermatomyositis (systemic angiopathy) of childhood . Medicine 45:261-289, 1966.Crossref 11. Oppenheim JJ: Relationship of in vitro lymphocyte transformation to delayed hypersensitivity in guinea pigs and man . Fed Proc 27:21, 1968.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Jan 1, 1972

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