An atypical cutaneous presentation of vasculitis with features of Churg-Strauss syndrome, associated with anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane antibodies.
We report the case of a 59-year-old woman who presented with a persistent papular and nodular cutaneous eruption and new-onset asthma, with normal renal function but persistent haematuria and proteinuria. Investigations revealed eosinophilia, both antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibodies on serological testing (double-positive vasculitis), and a focal necrotizing glomerulonephritis on renal biopsy. Histological examination of a skin biopsy showed a dense neutrophilic infiltrate with focal fibrinoid necrosis and few eosinophils. The clinical and pathological features suggested a double-positive vasculitis/Churg-Strauss overlap syndrome presenting with a predominantly neutrophilic dermatosis. Specific cutaneous features in patients with double-positive vasculitis have not been documented previously. The patient has responded extremely well to immunosuppressive treatment and her disease is currently in remission.
Gleeson
C M
CM
Department of Dermatology, Chelsea and Westminster NHS Foundation Trust, London, UK. cgleeson@doctors.org.uk
Levy
J B
JB
Cook
H T
HT
Francis
N D
ND
Robson
A
A
Pope
F M
FM
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism*; D001323:Autoantibodies / Q000378:metabolism*; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D004802:Eosinophilia / Q000473:pathology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D016896:Treatment Outcome; D014657:Vasculitis / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D007166:Immunosuppressive Agents; C051951:antiglomerular basement membrane antibody