Hypereosinophilic syndrome with various skin lesions and juvenile temporal arteritis

Abstract

Hypereosinophilic syndrome (HES) is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of the skin and many other organs. The commonest cutaneous features include erythematous pruritic maculopapules and nodules, angio-oedema or urticarial plaques. However, some case reports have indicated that eosinophilic cellulitis, cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may also occur as cutaneous features of HES. Juvenile temporal arteritis (JTA) of unknown cause is characterized by an asymptomatic nodule in the temporal artery area in young adults. Histologically, the lesion is characterized by a significant intimal thickening with moderate eosinophilic infiltrates, constriction or occlusion of the vascular lumen and absence of giant cells. We report a patient with HES presenting with eosinophilic cellulitis, Raynaud's phenomenon, digital gangrene and JTA. JTA may also be one of the features of HES.

Evaluation and Differential Diagnosis of Marked, Persistent Eosinophilia

“…Although mild eosinophilia is common, marked eosinophilia is rare in many of the other vasculitides but has been seen in patients with cutaneous necrotizing vasculitis, thromboangiitis obliterans with eosinophilia of the temporal arteritis [74] and in Wegener's granulomatosis. [75]…”

A Stab in the Dark: A Case Report of an Atypical Presentation of Giant Cell Arteritis (GCA)

“…Eosinophilia was a striking feature in JG and influenced extended serological testing. Although there is a link between hyper-eosinophilic syndrome and juvenile temporal arteritis [55], there are no strong clinical associations between peripheral blood eosinophilia and adult GCA [56]. The eosinophilia in our case may have simply reflected the general elevation in inflammatory cell milieu.…”

Concurrent bilateral juvenile temporal arteritis and hypereosinophilic syndrome: a case report and review of the literature

“…These patients complained of painless nodules on their temple, and the pathologic findings revealed nongiant cell inflammation of temporal arteries [4]. From then on until February 2019, according to our literature review through PubMed and Scopus, 18 more cases have been reported [23567891011121314151617181920]. Including the present case, a total of 23 cases of JTA were reported and their mean age was 27 years (range, 7 to 44 years) and there was the male predominance (18 men and 5 women).…”

“…In addition, one of the recurred cases reported that eosinophil count was elevated at recurrence [18]. Although only a small number of cases were available for the trend of eosinophil count after excision [8131819], eosinophilia may persist even if temporal arteritis improves without systemic steroids, and may lead to recurrence because documented recurrences occurred only in the patients with JTA with eosinophilia who treated locally. According to very recent case series (n = 12) from France, 41.7% (5 of 12) had peripheral eosinophilia, and this figure was similar to our estimate of 43.4% (10 of 23).…”

“…Since the first report of JTA in 1975 [4], a total of 22 cases have been reported so far [234567891011121314151617181920], but its etiology, pathogenesis, and long-term prognosis are still unknown. Although JTA is a localized disease, it is often accompanied by peripheral blood eosinophilia, so some authors have suggested the concept of JTA with eosinophilia [81213]. However, its association with pathogenesis, clinical features, and prognosis of JTA remains unclear.…”