Table 10 Hypereosinophilic syndromes

EGPA

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Sinus polyps, asthma, migratory pulmonary infiltrates, necrotizing glomerulonephritis, peripheral neuropathy (mononeuritis multiplex), cutaneous vasculitis and myocarditis

Peripheral blood eosinophilia, increased IgE. ANCA-p positivity, MPO positivity, PR-3 positivity (rare); Kidney biopsy: pauci-immune glomerulonephritis; Skin or lung biopsy: non-caseating granulomas, perivascular eosinophil infiltrates

Induction: CS or avacopan with CYC or RTX, PLEXˆ not routinely recommended; Remission: RTX, MEP, AZA, MMF

Eosinophilic fasciitis&

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Skin tightening, peau d’orange pattern, skin groove sign, prayer sign, muscle weakness; Benign or malignant hematologic abnormalities

Peripheral blood eosinophilia, hypergammaglobulinemia; Increased aldolase and CK levels; MRI: abnormal fascial signal intensity and enhancement; Deep skin biopsy: eosinophilic infiltrates with fasciitis

CS, MTX, CYC, AZA, cyclosporine and RTX

Eosinophilic myositis

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Pain and calf swelling (other muscles can be affected), proximal weakness

Peripheral blood eosinophilia, increased aldolase and CK levels; Biopsy: Deep mononuclear cell infiltration (eosinophilic or not), with muscle fiber invasion and necrosis on muscle biopsy

Spontaneously remission;

Relapsing: CS, NSAID

Kimura’s disease¶

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Painless head and/or neck lymphadenopathy or salivary gland enlargement (more common in Asian population); Allergic skin disease and glomerulonephritis has been associated in few cases

Peripheral blood eosinophilia, increased IgE; Biopsy: nodular, diffuse, and mixed inflammatory infiltrate composed mainly of lymphocytes and eosinophils, lymphoid follicles are hyperplastic and contain prominent germination centers

CS, radiotherapy, surgical resection

ALHE

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Dermal papules and nodules generally in head and neck region (more common in Asian population); Orbital region may be involved

Absent of peripheral blood eosinophilia, normal IgE serum levels; Biopsy: numerous enlarged endothelial cells in the lumen of an abnormal blood vessel; Presence of lymphocytes and eosinophils

Surgical resection; isotretinoin, laser, cryotherapy, imiquimod

PPP syndrome

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Panniculitis, arthritis and/or bone necrosis, acute or chronic pancreatitis or pancreatic tumors; Fever, abdominal pain, vomiting and nausea

Peripheral blood eosinophilia Biopsy: lobular non-vasculitis panniculitis

CS, NSAID;

Control the underlying pancreatic disease

RCNEV

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Annular urticarial plaques, purpuric papules, angioedema, chronic relapsing process; Absence of any features of systemic disease

Peripheral blood eosinophilia; Biopsy: necrotizing vasculitis of dermal small vessels with prominent eosinophilic infiltration; Granulomas and leukocytoclastic features are typically absent

CS, NSAID, AZA, MTX and tacrolimus

Strongyloidiasis

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Diarrhea, weight loss, epigastric pain, pneumonia, cutaneous periumbilical purpura, septic shock (hyperinfection syndrome)

Peripheral blood eosinophilia; Serologic detection of Strongyloides specific antibodies; Microscopic detection of larvae in stool with BM or APC culture or qPCR; Biopsy: microscopic detection of larvae in colonic tissue

Mild cases: ivermectin;

Severe: ivermectin + albendazole

Loeffler syndrome

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Dyspnea, cough, wheezing, pneumonia, more rarely myalgia, anorexia, hemoptysis and urticaria

Peripheral blood eosinophilia. BAL with increased eosinophil count; Serologic detection of Strongyloides specific antibodies; Microscopic detection of parasites larvae of A. lumbricoides or N. americanus or A. duodenale or S. stercoralis

Ivermectin or albendazole