[Pathogenesis and histomorphology of the so-called Omenn syndrome].
1. The Omen-syndrome is not a disease on its own, but a complication of congenital SCID. 2. In contrast to patients with classical SCID, patients with Omenn-syndrome possess mature T-cells, which are either of maternal or of host origin. 3. These T-cells are involved in the pathogenesis of the characteristic tissue changes, in particular of skin and lymph nodes (Langerhans-histiocytosis with eosinophilia). 4. The detection of immunodeficiency in Omenn-syndrome is difficult since the lymph nodes are enlarged in contrast to patients with classical SCID. The histomorphological analysis of lymph nodes in Omenn-syndrome is considerably complicated by secondary changes closely resembling dermatopathic lymphadenopathia.
Vossbeck
S
S
Abteilung II, Universitätskinderklinik Ulm.
Friedrich
W
W
Heymer
B
B
D004804:Eosinophils / Q000276:immunology; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology*; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000150:complications / Q000276:immunology* / Q000473:pathology*; D012867:Skin / Q000276:immunology / Q000473:pathology*; D013577:Syndrome; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*
D006680:HLA Antigens
Pathogenese und Histomorphologie des sogenannten Omenn-Syndroms.