Hypereosinophilic syndrome with various skin lesions and juvenile temporal arteritis.
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.
Ito
K
K
Department of Dermatology, Kawaguchi Medical Center, Saitama, Japan. ito_kei@jcom.home.ne.jp
Hara
H
H
Okada
T
T
Terui
T
T
D000328:Adult; D013700:Giant Cell Arteritis / Q000473:pathology*; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000473:pathology*; D007868:Leg Dermatoses / Q000473:pathology*; D008297:Male; D011537:Pruritus / Q000473:pathology