Chronic blepharitis and pyoderma of the scalp: an immune deficiency state in a father and son with hypercupremia and decreased intracellular killing.
An immune deficiency state is proposed as the cause of a disorder affecting a father and son with chronic dermatitis, purulent blepharitis with corneal ulceration, and scarring pyodermatous alopecia of the scalp. The results of immunologic investigation revealed abnormal neutrophil function with a variable decrease in intracellular killing, decreased lymphocyte transformation, increased serum IgG and IgE, and elevated serum copper levels. These findings will be compared with previously described immune deficiency disorders.
D000328:Adult; D001762:Blepharitis / Q000235:genetics*; D002648:Child; D002908:Chronic Disease; D003300:Copper / Q000097:blood; D004485:Eczema / Q000235:genetics; D005141:Eyelid Diseases / Q000235:genetics*; D006801:Humans; D007073:Immunoglobulin E / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000235:genetics*; D008213:Lymphocyte Activation; D008297:Male; D009504:Neutrophils / Q000276:immunology; D011711:Pyoderma / Q000235:genetics*; D012536:Scalp Dermatoses / Q000235:genetics*
D007074:Immunoglobulin G; D007073:Immunoglobulin E; D003300:Copper