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2016 ; 17
(ä): 864-868
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Eosinophilic Granulomatosis with Polyangiitis Presenting with Skin Rashes,
Eosinophilic Cholecystitis, and Retinal Vasculitis
#MMPMID27857032
Zeng M
; Liu X
; Liu Y
Am J Case Rep
2016[Nov]; 17
(ä): 864-868
PMID27857032
show ga
BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA), also known as
Churg-Strauss syndrome (CSS), is a rare vasculitis of unknown etiology. Most of
the patients have a long history of asthma and then develop autoimmune
inflammation of small and medium-sized blood vessels, with consequent reduction
of blood flow to various organs and tissues. EGPA can cause disorders in multiple
systems; the most seriously affected organs are the retina, kidney, brain,
cardiovascular system, and skin. CASE REPORT The patient was hospitalized for
high fever and skin rashes and then developed right upper abdominal pain,
decreased visual acuity, coma, and convulsions. Laboratory investigations showed
marked eosinophilia (9412/mm3). Following cholecystectomy, histopathological
examination revealed a marked inflammatory cell infiltrate composed mainly of
eosinophils. Retinal vasculitis and medium and peripheral vascular closure were
confirmed by fundus fluorescence angiography (FFA). The coma and convulsions were
controlled successfully by high-dose methylprednisolone. After gradual tapering
of the methylprednisolone, the patient's blood count recovered to a normal level,
and the other systematic disorders disappeared; however, she was left with
irreversible blindness. CONCLUSIONS EGPA can cause eosinophilic cholecystitis,
retinal vasculitis, and neuropathy in the short term and calls for effective
treatments in order to avoid binocular blindness.
|Biopsy
[MESH]
|Cholecystitis/diagnosis/*etiology
[MESH]
|Diagnosis, Differential
[MESH]
|Eosinophilia/diagnosis/*etiology
[MESH]
|Exanthema/diagnosis/*etiology
[MESH]
|Female
[MESH]
|Granulomatosis with Polyangiitis/complications/*diagnosis
[MESH]