A delayed diagnosis of eosinophilic granulomatosis with polyangiitis complicated with extensive artery occlusion of lower extremities in children: case report and literature review

Abstract

Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis in children. A delayed or missed diagnosis of pediatric EGPA is common, owing to the atypical clinical manifestation and limited recognition of this disorder. The vasculitis in EGPA typically involves small to medium size vessels. Extensive occlusion of arteries in the extremities was being extremely rare and has never been reported in children. Case presentation A 10-year and 10-month-old girl with recurrent wheezing and breathlessness during exercise, was initially diagnosed with asthma at the age of five years. Despite unexplained manifestations, including intermittent remarkably increased eosinophilia, uncontrolled allergic rhinitis, and recurrent petechia, from the onset of asthma through to its remission, the consideration of EGPA was completely ignored until the patient presented with aggravated petechia and severe ulceration of the lower extremities, associated with extensive stenosis and/or occlusion of the arteries of the shank and foot. Given her history of asthma, eosinophilia, allergic rhinitis, mononeuropathy, pulmonary infiltrates, and vasculitis confirmed by the skin biopsy, the diagnosis of EGPA was ultimately confirmed. Regrettably, the initial inappropriate and irrational use of corticosteroid failed to relieve the symptoms until more aggressive treatment with intravenous methylprednisolone was started. This was followed by methotrexate treatment, with tapering of prednisone, without relapse over a six-month follow-up. Conclusions Pediatric rheumatologists should be alert to the possibility of EGPA in children with refractory asthma associated with unexplained manifestations, and should be aware of the thromboembolic complications as vascular sequelae of EGPA.

Eosinophilic Granulomatosis with Polyangiitis in an 8-year-old Girl Manifesting as Hypereosinophilic Syndrome with Myocarditis, Stroke, and Subsequent Orbital Involvement

“…The prevalence and incidence of HES is unknown, but it is considered a rare disease in children and affected adults between 20 and 50 years old, with a 9:1 male-female ratio [12] and EGPA is a rare systemic vasculitis in children [16]. Statistical data and scientific publications show that HES and EGPA are exceedingly rare.…”

ANCA-Negative EGPA With Pulmonary, Cutaneous, and Neurological Manifestations in a 25-Year-Old Male: A Case Report

“…EGPA is a type of ANCA-associated vasculitis that predominantly affects small-and medium-sized vessels of many organs simultaneously [6]. Most common manifestations in children are seen in the respiratory tract (69%), cutaneous tissue (61-62%), gastrointestinal tract (46%), cardiovascular system (46%), paranasal sinus abnormality (38%), arthritis/arthralgia (38%), and peripheral nerves (15%) [7][8][9][10][11]. In adults, other respiratory features also come to observation while the overall pattern of involvement is almost similar to children.…”

Negative anti-neutrophil cytoplasmic antibodies and eosinophilic granulomatosis with polyangiitis accompanied by cough variant asthma: a case report

“…Some studies suggest that cutaneous manifestations are present in 40% to 81% of EGPA patients (16). It has been reported that the most common presenting features of EGPA in children are: pulmonary (69%), skin (61-64%), gastrointestinal (46%), and cardiac manifestations (46%); paranasal sinus abnormality (38%); arthritis/arthralgia (38%); neurological involvement (15%); and even vasculitis (15,(17)(18)(19). In another study of children, the respiratory system was found to be mostly involved, mainly the upper airways (85%), while other frequently involved organ systems were the skin (71%), digestive tract (64%), and heart (57%) (20).…”