Omenn's syndrome

International Journal of Dermatology

Volumn 36, Issue 2, 1997, Pages 141-144

  Nazzari, Guido ^b   Drago, Francesco ^a   Crovato, Franco ^b  

^a UNIVERSITY OF GENOA   (Italy)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

ARTICLE; CASE REPORT; COMBINED IMMUNODEFICIENCY; HUMAN; HUMAN TISSUE; IMMUNE DEFICIENCY; INFANT; MALE; RASH; SKIN BIOPSY;

EID: 0030989780     PISSN: 00119059     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1365-4362.1997.tb03077.x     Document Type: Article

References (19)

1. Omenn GS. Familial reticuloendotheliosis with eosinophilia. N Engl J Med 1965; 273: 427-432.
2. Barth RF, Vergera GG, Khurana SK, et al. Rapidly fatal familial histiocytosis associated with eosinophilia and primary immunological deficiency. Lancet 1972; i: 503-506.
3. Ruco LP, Stoppacciaro A, Pezzella F, et al. The Omenn's syndrome: histological, immunohistochemical and ultrastructural evidence for a partial T cell deficiency evolving in an abnormal proliferation of T lymphocytes and S-100+/T6+ Langerhans-like cells. Virchows Arch (A) 1985; 407: 69-82.
4. Glastre C, Rigal D. Le syndrome d'Omenn. Pédiatrie 1990; 45: 301-305.
5. Karol RA, Eng J, Cooper JB, et al. Imbalances in subsets of T lymphoctyes in an inbred pedigree with Omenn's syndrome. Clin Immunol Immunopathol 1983; 27: 411-427.
6. Saint-Basile G, Le Deist F, Villartay JP, et al. Restricted heterogeneity of T lymphocytes in combined immunodeficiency with hypereosinophilia (Omenn's syndrome). J Clin Invest 1991; 87: 1352-1359.
7. Businco L, Di Fazio A, Ziruolo MG, et al. Clinical and immunological findings in four infants with Omenn's syndrome: a form of severe combined immunodeficiency with phenotypically normal T cells, elevated IgE, and eosinophilia. Clin Immunol Immunopathol 1987; 44: 113-133.
8. Le Deist F, Saint Basile G, Mazerolles F, et al. Primary membrane T cell immunodeficiencies. Clin Immunol Immunopathol 1991; 61: S56-60.
9. Wirt DP, Brooks EG, Vaidya S, et al. Novel T lymphocyte population in combined immunodeficiency with features of graft-versus-host-disease. N Eng J Med 1989; 321: 370-374.
10. Pupo RA, Tyring SK, Raimer SS, et al. Omenn's syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants with persistent erythroderma and failure to thrive. J Am Acad Dermatol 1991; 25: 442-446.
11. Wyss M, Fliedner V, Jacot-Des-Combes E, et al. A lymphoproliferative syndrome, "Cutaneous Dystrophy" and combined immune deficiency with lack of helper T-cell factor. Clin Immunol Immunopathol 1982; 23: 34-49.
12. Ochs HD, Davis SD, Mickelson E, et al. Combined immunodeficiency and reticuloendotheliosis with eosinophilia. J Pediatr 1974; 85: 463-465.
13. Junker AK, Chan WK, Massing BG. Clinical and , immune recovery from Omenn syndrome after bone marrow transplantation. J Pediatr 1989; 114: 596-600.
14. Akhter J, Krantman HJ, Hopper J, et al. Lymphoproliferation following antigenic stimulation in severe combined immunodeficiency disease. Clin Immunol Immunopathol 1981; 20: 361-372.
15. Tachinami T, Koizumi S, Yachie A, et al. Immune status in two brothers with Omenn's syndrome: no discernible chimerism on FACS analysis using a monoclonal antibody specific for a maternally restricted HLA antigen. Am J Pediatr Hematol Oncol 1990; 12: 343-350.
16. Cohen A, Mansour A, Dosch HM, et al. Association of a lymphocyte purine enzyme deficiency (5′ nucleotidase) with combined immunodeficiency. Clin Immunol Immunopathol 1980; 15: 245-250.
17. Jouan H, Le Deist F, Nezelof C. Omenn's syndrome -pathologic arguments in favor of a graft versus host pathogenesis: a report of nine cases. Hum Pathol 1987; 18: 1101-1108.
18. Heyderman RS, Morgan G, Levinsky RJ, et al. Successful bone marrow transplantation and treatment of BCG infection in two patients with severe combined immunodeficiency. Eur J Pediatr 1991; 150: 477-480.
19. Bruckmann C, Lindner W, Roos R, et al. Severe pulmonary vascular occlusive disease following bone marrow transplantation in Omenn's syndrome. Eur J Pediatr 1991; 150: 242-245.