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Therapeutic Approach

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 129-1Erythema Elevatum Diutinum Treatment Table

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