Peripheral gangrene in adult‐onset Kawasaki disease

Abstract

Kawasaki disease (KD) is an acute vasculitis characterized by marked tropism of the coronary vessels. Usually a childhood disease, KD can occasionally be observed in adults. We report a case of adult-onset KD that presented as a prolonged fever of unknown origin with subsequent development of severe vasculitis, manifested by coronary aneurysms and peripheral gangrene of the lower limbs. Therapy with intravenous immunoglobulins, corticosteroids, aspirin, anticoagulants, and prostacyclin analogue resulted in rapid improvement in the patient's condition but he required partial distal amputation for irreversible gangrene. Peripheral ischaemia leading to gangrene is a very rare feature of KD vasculitis with only 19 cases previously published in the Medline-indexed literature. The outcomes of those patients were poor, being either fatal or requiring distal amputation. All prior cases affected young children less than 1 year old and, to the best of our knowledge, the present case is the first description of peripheral gangrene in adult-onset KD.

Adult Kawasaki disease in a European patient: a case report and review of the literature

“…Susceptibility to coronary artery lesions formation has been associated in various populations with specific single nucleotide polymorphisms in a wide range of genes, such as ITPKC [ 19 ], IL-10 [ 28 ], TGFBR2 [ 29 ], DC-SIGN [ 30 ] and KCNN2 [ 31 ]. Arteries other than the coronary ones are also affected by the disease: a hepatic artery aneurysm [ 32 ], a left humeral artery aneurysm, with absence of flow in the antebrachial arteries causing a peripheral gangrene [ 33 ], a proximal gastroduodenal artery occlusion, and a mildly irregular splenic artery associated with obstruction of the distal intrasplenic branches [ 34 ] are described in the literature.…”

“…Clinical and laboratory findings concerning liver inflammation are jaundice [ 32 , 40 , 48 , 49 ], hepatomegaly [ 15 , 32 , 50 ], right upper quadrant pain [ 32 , 39 ], cholestatic hepatitis [ 6 , 44 , 46 - 49 , 51 ], hepatic artery aneurysm [ 32 ], elevated aspartate and alanine aminotransferase levels [ 2 , 8 , 11 , 13 , 33 , 34 , 40 , 41 , 43 , 52 , 53 ] and low albumin levels [ 11 , 40 , 53 ].…”

Kawasaki disease complicated by peripheral gangrene and a ventricular septal defect: An unusual association

“…Systemic examination did not reveal any other positive signs. The laboratory examinations were as follows: Hb 8.1 g/dl; WBC 32,100/mm 3 ; platelet 2,91,000/ mm 3 ; ESR 81 mm/h; CRP 19.5 mg/dl; Albumin 3 g/dl and SGPT 70 IU/L. The child was admitted to the pediatric intensive care unit (PICU) and broad spectrum antibiotics were started.…”

“…Kawasaki disease (KD) is an acute illness of unknown cause that chiefly affects infants and children and is characterized especially by fever, rash, conjunctivitis, inflammation of lips and tongue, and unilateral swollen lymph nodes of the neck and is the leading cause of acquired heart disease. [1][2][3] KD is associated with systemic vasculitis particularly affecting the coronary arteries, causing coronary artery aneurysms (CAA) in 15-25% of untreated patients while 2-3% of untreated cases die as a result of coronary vasculitis. [4][5][6] Peripheral gangrene is a known but rare complication of KD that occurs almost exclusively in infants.…”

Kawasaki disease in adults: Observations in France and literature review

“…Most of the incomplete cases were diagnosed by the presence of coronary vasculitis. A few cases were retrospectively diagnosed after MI complicating coronary aneurysm [26,33], as observed in this series.…”