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Erythema elevatum diutinum
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Erythema elevatum diutinum (EED) is a rare neutrophilic dermatosis consisting of violaceous, brown or red papules, plaques, nodules, and occasionally vesicobullous lesions over the extensor surfaces of the joints and buttocks, genitalia, trunk, and face. Early lesions tend to be soft and erythematous. Advanced lesions tend to be nodular and firm, secondary to fibrosis. EED is thought to be a form of immune complex–mediated leukocytoclastic vasculitis, although its etiology remains unclear. Infections (including HIV and streptococcal), hematologic abnormalities, inflammatory bowel disease, celiac disease, verrucous carcinoma, systemic lupus erythematosus, primary Sjögren syndrome, ophthalmic disorders (peripheral keratitis), and pulmonary lymphoepithelioma-like carcinoma (possibly as part of a paraneoplastic syndrome) have been associated. Drug-induced EED may result from interferon-β, erythropoietin, antituberculosis chemotherapy, and cisplatin exposure.
Differential diagnoses of early lesions of EED include extrafacial granuloma faciale, Sweet syndrome, rheumatoid neutrophilic dermatitis or palisaded neutrophilic and granulomatous, dermatitis, and advanced lesions include rheumatoid nodules and multicentric reticulohistiocytosis.
Management Strategy
EED is a chronic disease that may persist for several years or decades and typically proves challenging to treat.
After management of any underlying disease, dapsone 100 mg daily remains the initial treatment of choice. The response may be partial and dose dependent. Both intralesional and systemic corticosteroids (prednisolone 30–40 mg daily) have produced favorable responses.
Sulfonamides (sulfamethoxypyridazine 500 mg once daily and sulfapyridine 0.5–1 g three times daily), nicotinamide/niacinamide 100 mg three times daily, colchicine 0.5 mg twice daily with 0.5 mg three times daily for 3–4 days to abate minor disease flares, and chloroquine 300 mg daily have produced resolution of lesions.
Specific Investigations
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Erythema elevatum diutinum: clinical, histopathologic, and immunohistochemical characteristics of six patients
Wahl CE, Bouldin MB, Gibson LE. Am J Dermatopathol 2005; 27: 397–400.
The vascular endothelium of EED stains positive for CD31, CD34, VEGF, and factor VIII, and negative for factor XIIIa, TGFβ, and LANA. This pattern does not distinguish it from similar-appearing lesions. Therefore, the chronic and recurrent nature of EED is the primary means of distinguishing it from entities that are clinically and histologically similar.
Erythema elevatum diutinum and IgA paraproteinaemia: “a preclinical iceberg”
Chowdhury MMU, Inaloz HS, Motley RJ, et al. Int J Dermatol 2002; 41: 368–70.
The technique of immunofixation electrophoresis is more sensitive than immunoelectrophoresis. This uses a combination of zone electrophoresis and immunoprecipitation with specific antisera to detect monoclonal immunoglobulins or light chains at very low concentrations in serum and urine. This is useful in EED because patients may have associated paraproteinemias, which, in some cases, may undergo malignant transformation.
The authors note that in monoclonal disorders there is extensive asymptomatic tumor proliferation and possible malignant transformation in 20% of patients during long-term follow-up. It was recommended that there should be lengthy follow-up and monitoring for patients with both EED and IgA paraproteinemia because of the risk of progression to IgA myeloma.
Is IgA antineutrophil cytoplasmic antibody a marker for patients with erythema elevatum diutinum? A further three cases demonstrating this association
Crichlow SM, Alexandroff AB, Simpson RC, et al. Br J Dermatol 2011; 164: 675–7.
Two studies evaluating the prevalence of ANCAs in EED. IgA ANCAs were present in all patients with EED.
Erythema elevatum diutinum and HIV infection: a report of five cases
Muratori S, Carrera C, Gorani A, et al. Br J Dermatol 1999; 141: 335–8.
The largest case series of patients with EED and HIV infection. Streptococcal infection seemed to trigger exacerbations in four of five patients. EED can stimulate Kaposi sarcoma and bacillary angiomatosis, which can be challenging in the context of an HIV-seropositive patient. Histopathologic confirmation of the diagnosis is therefore advocated.
Erythema elevatum diutinum associated with peripheral ulcerative keratitis
Vaiyavatjamai P, Wattanakrai P. J Eur Acad Dermatol Venereol 2011; 25: 741–2.
Progressive keratolysis with pseudopterygium associated with erythema elevatum diutinum
Lekhanont K, Patarakittam T, Mantachote K, et al. Ophthalmology 2011; 118: 927–33.
Erythema elevatum diutinum with verrucous carcinoma: a rare association
Nair SR, Viswanath V, Sonavane AD, et al. Indian J Dermatol Venereol Leprol 2010; 76: 420–2.
Erythema elevatum diutinum as a paraneoplastic syndrome in a patient with pulmonary lymphoepithelioma-like carcinoma
Liu T-C, Chen I-S, Lin T-K, et al. Lung Cancer 2009; 63: 151–3.
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