ERYTHEMA ELEVATUM DIUTINUM
Erythema elevatum et diutinum (EED) is a rare, chronic dermatosis characterized as a leukocytoclastic vasculitis. Early cutaneous findings include red-brown, violaceous papules, and plaques on extensor surfaces and acral sites, later developing into firm nodules and indurated masses.
EED has been associated with extracutaneous findings, including arthralgias, scleritis, panuveitis, peripheral ulcerative keratitis, oral and penile ulcers, and neuropathy. The presence of such extracutaneous manifestations in EED patients suggests that it may be a multiorgan entity.1 Additionally, EED may be incited by various systemic diseases, including infections, autoimmune diseases, and hematologic malignancies. Frequently cited infections include HIV, β-hemolytic streptococci, HBV, tuberculosis, and syphilis.2 Autoimmune diseases found to be associated with EED include inflammatory bowel disease, celiac disease, SLE, and RA, and notable hematologic malignancies include IgA paraproteinemia, monoclonal gammopathy, and myelodysplasias.
Dapsone, an antimicrobial that impairs neutrophil chemotaxis and function, is the treatment of choice for EED.3,4 Treatment with dapsone, however, may not be as effective in HIV-positive patients and for patients with late stage fibrotic lesions.5 Antiretroviral therapy is typically added to the treatment regimen for HIV-positive patients. Other treatment options consist of anti-inflammatory, antimicrobial, and immunosuppressive agents.2,6 Additionally, for fibrotic nodules that respond poorly to dapsone, surgical excision is considered if the extent of lesions is limited.1 Treatment of an associated underlying disorder is also recommended.7 See Table 129-1.
Table Graphic Jump Location Table 129-1Erythema Elevatum Diutinum Treatment Table ||Download (.pdf) Table 129-1 Erythema Elevatum Diutinum Treatment Table
|MEDICATION NAME ||INDICATION ||MECHANISM OF ACTION ||DOSING ||ADVERSE EFFECTS ||SUGGESTED MONITORING ||LEVEL OF EVIDENCE (REFERENCE) |
|Dapsone ||Treatment of choice. First-line EED treatment.7 A single case report also showed benefits of topical 5% dapsone gel prior to start of oral therapy with improvement in erythema and flattening of lesions.3 ||Inhibits bacterial synthesis of dihydrofolic acid, via competition with aminobenzoate for the active site of dihydropteroate synthase, thereby inhibiting nucleic acid synthesis. ||50 mg-300 mg7 (start at 25 mg-50 mg/d with maintenance dose of 100 mg daily) ||Hemolysis, agranulocytosis, methemoglobinemia, dapsone hypersensitivity syndrome, and peripheral neuropathy || |
Baseline: G6PD level, CBC/reticulocyte count, CMP.
Monitoring: CBC/reticulocyte count qwk ×4, then qmo ×6, then q6mo; LFTs q3-4mo.
|Diclofenac sodium ||First-line EED treatment ||The 6 major chemical classes of NSAIDs all have the common property of inhibiting cyclooxygenase ||100 mg/d ||Gastrointestinal effects. anemia, renal compromise, and drug-related liver injury || ||III2,6 |
|Intralesional Corticosteroid ||First-line EED treatment ||Anti-inflammatory depending on medication utilized || |
The concentration of injection vary according to lesion type and site.
Severe cases may require 0.5-1 mg/kg/d of systemic prednisone equivalents with slow taper.
|Atrophy, hypopigmentation, and rarely, sterile abscess formation ||Oral steroids are rarely indicated and, therefore, no risk for serious adverse effects associated with long-term steroid therapy ||III7,27...|