Churg‐Strauss Syndrome (CSS) Manifested as Necrosis of Fingers and Toes and Liver Infarction

Abstract

We report a case of Churg-Strauss syndrome (CSS) with necrosis of the fingers and toes and liver infarction. A 59-year-old man with asthma suddenly noticed that his fingers and toes felt unusually cold. This condition worsened progressively, and some digits became necrotic within several weeks. Laboratory studies revealed hypereosinophilia and an extremely elevated serum level of IgE. Digital subtraction angiography of the extremities revealed extensive irregular narrowing of small and medium-sized arteries in the extremities. Abdominal computed tomography (CT) revealed an area of low density at the periphery of the right lobe of the liver. Angiography revealed irregular narrowing of small arteries that corresponded to the ischemic area. A nerve conduction study suggested sensory nerve neuropathy. The preceding asthma, acute onset of digital necrosis, liver infarction, neuropathy, and hypereosinophilia strongly suggested a diagnosis of CSS. The patient was treated with 40 mg of prednisolone and 120 micro g of intravenous prostaglandin E1 daily, and all the digits that had turned black and necrotic were amputated. After the amputation, the dose of prednisolone was gradually reduced, and no new lesions appeared on the skin or in the liver. The rare possible complications of CSS, including necrosis of digits and liver infarction, should not be ignored.

Clinicopathological Conference: A 38‐year‐old Man with Respiratory Failure—The Case of the Underground Illness

“…Acute hypersensitivity pneumonitis occurs within hours of exposure and should resolve spontaneously within 12-72 hours, which does not fit the clinical picture for our patient. 4 Churg-Strauss angiitis is a necrotizing angiitis that occurs in patients with asthma, 5 neither of which our patient has. Eosinophilic granulomatosis in adults occurs mostly in smokers, 6 and our patient does not smoke.…”

Churg–Strauss syndrome manifesting as cholestasis and diagnosed by liver biopsy

“…However, this was not likely in the present patient, because the inflammation around the hepatic artery was demonstrated by the liver biopsy and the expression of MRP2 was normal. Digital subtraction angiography revealed irregular narrowing of the small arteries in the liver in a patient with Churg–Strauss syndrome 4 . A recent study demonstrated eosinophilic infiltrates in vessels in the portal area of the liver in a patient with Churg–Strauss syndrome 5 .…”

“…The symptoms are palpable cutaneous purpura, mononeuritis multiplex, gastrointestinal involvement and renal involvement. Liver involvement is rare, however, some studies described liver function abnormalities 4,5,7 . Severe systemic inflammation affects liver function, especially various transporters and tight junction proteins of hepatocytes 8–10 .…”

“…Churg–Strauss syndrome can affect any organ system in the body and the symptoms include neurological, nasal, pulmonary, cardiac, renal, gastrointestinal, vascular and skin manifestations 3 . However, hepatic manifestations are rather rare 4,5 . Here, we report a patient with Churg–Strauss syndrome diagnosed by liver biopsy who visited our hospital due to fever and liver dysfunction without hypereosinophilia or any other major manifestation except asthma.…”

Churg–Strauss syndrome manifested by appendicitis, cholecystitis and superficial micronodular liver lesions—an unusual clinicopathological presentation: Figure 1

“…Rare cases of intestinal erosion,4 ulceration and perforation3 7 8 have been described. Others include acalculous cholecystitis associated with large liver abscesses,4 liver infarction with cutaneous necrosis,5 and duodenitis 6. One case of oophoritis, complicating interpretation of the abdominal signs in women, has also been documented 9…”