We describe the clinicopathologic features of a 56-year-old woman affected
with Churg-Strauss syndrome with major peripheral nerve involvement. The pa
tient presented with a 1-month history of mainly distal upper-limb symmetri
cal paresthesias and hypostenia (bilateral "wrist drop"), palpable purpura
and eosinophilia. Multiple pulmonary infiltrates and asthma had been presen
t since the age of 52. Skin biopsy demonstrated an eosinophilic necrotizing
vasculitis. During the hospitalization she was submitted to cardiac, bronc
hopulmonary, renal, and gastrointestinal evaluation and EMG. Peripheral ner
ve and skeletal muscle biopsies were performed. Sural nerve biopsy showed a
marked degree of demyelination. A perivascular cellular infiltrate within
the epineurium was immunoreactive for T lymphocytes and macrophages. Strong
HLA-DR immunostaining was present in the endoneurium. IgM, IgE and fibrino
gen deposition was found in some epi-and endoneurial vessels. Muscle biopsy
showed neurogenic changes and 1 thrombosed vessel surrounded by mononuclea
r cells. Membrane attack complex (MAC) deposition was present in a few capi
llaries and major histocompatibility complex products I (MHCP I) was expres
sed at the subsarcolemmal level in a few isolated perivascular muscle fiber
s. After immunosuppressive therapy, the patient showed progressive improvem
ent of both clinical symptoms and neurophysiological parameters.