Published on 18/03/2015 by admin
Filed under Dermatology
Last modified 18/03/2015
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Pamela Fiandeiro, Emma Benton and Ian Coulson
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. EED is thought to be a form of immune complex-mediated vasculitis, although its etiology remains unclear. Infections (including HIV and streptococcal), hematologic abnormalities, autoimmune diseases, and other conditions have been associated.
EED is a chronic disease; there are only a few well-documented instances of spontaneous long-term resolution.
Dapsone 100 mg daily remains the initial treatment of choice. The response may be partial and dose dependent. Corticosteroids have also been effective in patients with EED. Topical betamethasone and topical fluocinolone acetonide have been used under occlusion with good effect. In other patients 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 100 mg three times daily, colchicine 0.5 mg twice daily with 0.5 mg three times daily for 3 to 4 days to abate minor disease flares, and chloroquine 300 mg daily have produced resolution of lesions.
EED has been reported in association with diseases such as HIV, hematological disorders, inflammatory bowel disease, celiac disease, verrucous carcinoma, systemic lupus erythematosus, primary Sjögren syndrome, ophthalmic disorders (peripheral keratitis), pulmonary lymphoepithelioma-like carcinoma (possibly as part of a paraneoplastic syndrome) and evidence of these should be sought. Drug induced EED may result from interferon-β, erythropoietin, antituberculosis chemotherapy, and cisplatin exposure.
Full blood count
Immunoglobulins and serum electrophoresis/ immunofixation electrophoresis
Antineutrophil cytoplasmic antibodies
Antireticulin and antigliadin antibodies
HIV risk factors enquiry
Histology
Lee AY, Nakagawa H, Nogita T, Ishibashi Y. J Cutan Pathol 1989; 16: 211–17.
An electron microscopic study of a patient with EED demonstrating that the characteristic histopathologic changes in early lesions include leukocytoclastic vasculitis and massive dermal infiltrate of neutrophils, histiocytes/macrophages, and Langerhans cells. In late lesions the inflammatory infiltrate is replaced by fibrosis with a dermal infiltrate of lymphocytes, histiocytes/macrophages, and Langerhans cells.
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.
Yiannias JA, El-Azhary RA, Gibson LE. J Am Acad Dermatol 1992; 26: 38–44.
Of 13 patients with EED, six had associated hematologic abnormalities, with IgA monoclonal gammopathy occurring most frequently.
Chowdhury MMU, Inaloz HS, Motley RJ, Knight AG. Int J Dermatol 2002; 41: 368–70.
The technique of immunofixation electrophoresis is more sensitive than immunoelectrophoresis and 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.
Crichlow SM, Alexandroff AB, Simpson RC, Saldanha G, Walker S, Harman KE. Br J Dermatol 2011; 164: 675–7.
Two studies evaluating the prevalence of antineutrophil cytoplasmic antibodies (ANCAs) in EED. IgA ANCAs were present in all patients with EED.
Muratori S, Carrera C, Gorani A, Alessi E. 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’s sarcoma and bacillary angiomatosis, which may be particularly confusing in the context of an HIV seropositive patient. Histopathological confirmation of the diagnosis is therefore advocated.
Vaiyavatjamai P, Wattanakrai P. J Eur Acad Dermatol Venereol 2011; 25: 741–2.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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