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Chapter 25: Eosinophilic Diseases

Kentaro Izumi; Hideyuki Ujiie

EOSINOPHILIC DISEASES

Wells Syndrome

Therapeutic Approach

Wells syndrome is a rare inflammatory skin disorder characterized clinically by pruritic erythematous plaques resembling cellulitis and histologically by dermal edema, eosinophil infiltration, and “flame figures.” Lesions usually improve drastically after systemic glucocorticoid administration. Recurrent or persistent cases can be effectively maintained with the administration of low-dose prednisone every other day. In refractory cases, minocycline, dapsone, griseofulvin, and antihistamines may be beneficial. Cyclosporine and IFN-α have also been used with success. For the treatment of localized or mild disease, medium- to high-potency topical corticosteroids may be sufficient. It also can be beneficial to treat the underlying condition or triggering factor, if identified. See Table 25-1.

Table 25-1Wells Syndrome Treatment Table
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Table 25-1 Wells Syndrome Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Corticosteroids Localized, mild, children Anti-inflammatory 0.1% mometasone furoate cream; apply QD Atrophy, telangiectasia, striae, acneiform eruption, allergic sensitization, purpura, changes in pigmentation, hypertrichosis None III1,2
Systemic Therapy
Corticosteroids Moderate to severe, refractory, recurrent Anti-inflammatory

0.5-2 mg/kg/d

5 mg every other day

Contraindicated in administration of live or live attenuated virus vaccines, systemic fungal infection

Adrenal suppression, immunosuppression, Kaposi sarcoma, myopathy, psychiatric disturbances, diabetes, hypertension, gastrointestinal ulcers, cataracts, glaucoma

 Blood pressure, weight, serum glucose, electrolytes, growth in pediatric patients, presence of infection, bone mineral density, assessment of hypothalamic–pituitary–adrenal axis suppression (eg, ACTH stimulation test, morning plasma cortisol test, urinary free cortisol test), Hgb, occult blood loss, chest X-ray, eye examination III2-4
Mepolizumab

Refractory

Recurrent

 Anti-inflammatory, reduction of eosinophil proliferation and survival by inhibition of IL-5 signaling subcutaneous dose of 100 mg every 4 wk Hypersensitivity, injection site reaction, headache, pruritus, rash, infection, diarrhea, abdominal pain, arthralgia None IV5
Omalizumab Refractory, recurrent Anti-inflammatory, reduction of free IgE, downregulation of FcεRI Subcutaneous dose of 300 mg every 4 wk Cerebrovascular events, fever/arthralgia/rash, hypersensitivity/anaphylactoid reactions Anaphylactic/hypersensitivity reactions, baseline serum total IgE, signs of infection IV6
Cyclosporine Recurrent Regulation of T-cell proliferation and cytokine production (IL-4, IL-5) Daily dose of 1.25-2.5 mg/kg (100-200 mg)

Contraindicated in abnormal renal function, uncontrolled hypertension, or malignancies

Gingival hyperplasia, hepatotoxicity, hyperkalemia, hypertension, hyperuricemia, infections, malignancy, nephrotoxicity, neurotoxicity, skin cancer, thrombotic microangiopathy

 Plasma concentrations, blood pressure, CBC, serum creatinine, BUN, uric acid, potassium, lipids, magnesium, liver function IV7
Dapsone Recurrent Inhibition of eosinophilic peroxidase and mast cell degranulation 50 mg/d Blood dyscrasias, serious dermatologic reactions, toxic hepatitis, cholestatic jaundice, hyperbilirubinemia, peripheral neuropathy, sulfonamide allergy, superinfection CBC, liver function, jaundice, hemolysis, blood dyscrasias IV8
Azathioprine Refractory to systemic steroid alone, recurrent Suppression of immune reaction n/a GI toxicity, hematologic toxicity (pancytopenia), hepatotoxicity, malignancy, infection, progressive multifocal leukoencephalopathy CBC, total bilirubin, liver function, creatinine clearance, infection, malignancy III9
Griseofulvin Recurrent Antifungal n/a

Contraindicated in hepatic failure, porphyria, pregnancy

Leukopenia, jaundice, elevated liver ...

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