Granulocyte activation in bullous diseases: release of granular proteins in bullous pemphigoid and pemphigus vulgaris.
BACKGROUND
Eosinophil and polymorphonuclear granulocytes may be involved in the formation of blisters in bullous dermatoses, particularly bullous pemphigoid.


OBJECTIVE
Our purpose was to evaluate the role of granulocyte activation in the pathogenesis of pemphigus vulgaris and bullous pemphigoid.


METHODS
Levels of eosinophil cationic protein (ECP) and neutrophil-derived myeloperoxidase (MPO) in blister fluid and serum and levels of serum IgE were determined in patients with bullous pemphigoid (n = 12), those with pemphigus vulgaris (n = 9) and healthy volunteers (n = 12).


RESULTS
In blister fluid and serum of patients with bullous pemphigoid, significantly elevated concentrations of ECP, MPO and IgE were detected as compared with controls. In contrast, ECP, MPO, and IgE levels in blister fluid and serum of patients with pemphigus vulgaris did not significantly differ from controls. Moreover, the MPO/ECP ratio in serum of patients with bullous pemphigoid was significantly decreased as compared with controls, whereas the MPO/ECP ratio in pemphigus vulgaris did not differ from controls, indicating a preferential activation of eosinophils in bullous pemphigoid only. In patients with bullous pemphigoid, serum levels of ECP and MPO significantly decreased during immunosuppressive therapy to levels similar to those of controls.


CONCLUSIONS
Activated granulocytes, releasing their granular contents such as ECP and MPO, may be of importance for blister formation in bullous pemphigoid and may be useful for monitoring disease activity.
Department of Dermatology, University of Freiburg, Germany.
W
W
Czech
J
J
Schaller
E
E
Schöpf
A
A
Kapp
D000368:Aged; D000369:Aged, 80 and over; D001798:Blood Proteins / Q000032:analysis; D047091:Eosinophil Granule Proteins; D004804:Eosinophils / Q000276:immunology*; D005260:Female; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood; D008297:Male; D008875:Middle Aged; D009504:Neutrophils / Q000276:immunology*; D010391:Pemphigoid, Bullous / Q000276:immunology*; D010392:Pemphigus / Q000276:immunology*; D009195:Peroxidase / Q000378:metabolism; D012260:Ribonucleases
D001798:Blood Proteins; D047091:Eosinophil Granule Proteins; D007073:Immunoglobulin E; D009195:Peroxidase; D012260:Ribonucleases