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Presentation

Elevated IgA-Associated Erythema Elevatum Diutinum Case Presentation and Treatment

Description

This case presentation described a 71-year-old man with a year-long history of multiple firm, mostly asymptomatic nodules on the lower extremities, including toes, heels, knee, and medial malleolus, with some overlying petechial macules. Initial differential diagnoses included infection, vasculitis, granulomatous disease, lymphoma, and histiocytic or vascular processes. Biopsies showed leukocytoclastic vasculitis with fibrinoid necrosis, neutrophils, cariorrhexis, histiocytes, and concentric fibrosis, while infectious stains were negative, supporting erythema elevatum diutinum (EED), a rare chronic small-vessel vasculitis. The speaker reviewed EED’s typical clinical features, pathogenesis, and associations, emphasizing that IgA monoclonal gammopathy is the most common, though infections and autoimmune diseases are also linked. In this patient, workup was negative except for elevated polyclonal IgA, so the diagnosis was idiopathic EED. Dapsone was identified as the best initial treatment, with steroids less effective and other options such as colchicine, tetracyclines, niacinamide, and excision considered later-line. The patient improved gradually on dapsone, dose escalation, and intralesional steroid injections, with flattening and fading of lesions over a year.

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Conclusions

The case is most consistent with erythema elevatum diutinum, a rare chronic small-vessel vasculitis characterized by firm red-brown to violaceous papules, plaques, or nodules.
Histology showing leukocytoclastic vasculitis with fibrinoid necrosis and concentric fibrosis supports the diagnosis of EED over infection, neoplasm, or granulomatous disease.
EED is most often associated with IgA gammopathy, but it can also be linked to infections such as streptococcal disease, hepatitis B, and HIV, as well as autoimmune disorders like rheumatoid arthritis.
A negative infectious and autoimmune workup with elevated polyclonal IgA supports an idiopathic or inflammation-associated form of EED rather than an overt systemic trigger.
Dapsone is the preferred initial treatment for EED and is usually more effective than oral corticosteroids.
Many patients require prolonged therapy, and relapse after stopping treatment is common, so management may need to be long-term.
Treating an underlying associated disorder or infection is important when one is found.
The patient improved clinically with higher-dose dapsone and intralesional corticosteroid injections, suggesting that EED lesions can respond well to medical therapy.
The presentation emphasizes that EED may precede the diagnosis of associated hematologic disease by years, so ongoing surveillance is warranted even when initial testing is negative.

Sharma V, et al. Indian J Dermatol Venereol Leprol 2013.
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