Eosinophilic Granulomatosis with Polyangiitis Presenting with Skin Rashes, Eosinophilic Cholecystitis, and Retinal Vasculitis
Challenging differential diagnosis, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)
Mingbing Zeng, Xialin Liu, Yizhi Liu
Zhongshang Ophthalmic Center, Sun Yat-sen University, Guangzhou, Guangdong, China (mainland)
Am J Case Rep 2016; 17:864-868
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.
Keywords: Cholecystitis, Microscopic polyangiitis, Retinal Vasculitis