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My diagnostic blind spot: Erythema elevatum diutinum

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By Warren R. Heymann, MD
Feb. 15, 2018

Erythema elevatum diutinum
Erythema elevatum diutinum depicted by erythematous-to-violaceous papules and plaques affecting the extensor surfaces of the patient's arms, legs, and buttocks.
Credit: JAAD

I’m certain that every dermatologist — myself included — has a diagnostic blind spot. I came to this realization during the first few years of our training program — each resident would consistently miss an obvious diagnosis throughout his or her training. One resident, an extraordinary clinician, had difficulty recognizing lichen planus, another resident herpes zoster, yet another couldn’t diagnose an id reaction, and so on. Personally, I always struggle with the diagnosis of erythema elevatum diutinum (EED). To make life more challenging, over the past year, several atypical variants of EED have been described.

EED is a rare, chronic dermatosis usually appearing as red–violet to red–brown papules, plaques, and nodules favoring extensor surfaces. Lesions often are asymptomatic, although patients may experience pain or a burning sensation. Extracutaneous symptoms include arthralgia, fever, or other constitutional symptoms. EED may be associated with infections (group B streptococci, HIV, syphilis, and viral hepatitis), hematologic abnormalities (IgA monoclonal gammopathy, myelodysplasia, myeloproliferative disorders, paraproteinemia, and hairy cell leukemia), autoimmune diseases (celiac disease, Crohn disease, systemic lupus erythematosus and rheumatoid arthritis), or other disorders. The histology varies with time; early lesions are those of a leukocytoclastic vasculitis, becoming more of a mixed infiltrate with ultimate fibrosis and potential extracellular cholesterol deposition. The etiology is a presumed immune-complex driven vasculitis. Dapsone is the most effective therapeutic agent for early lesions; other options include NSAIDs, niacinamide, tetracyclines, chloroquine, colchicine, and plasmapheresis. Topical and intralesional corticosteroids may be useful in mild cases, but systemic corticosteroids are rarely indicated. Spontaneous resolution often occurs after 5 to 10 years (1), but remember, “diutinum” is Latin for “long lasting.”
EED in atypical sites may hinder diagnosis. Keyal et al reported the case of a 51-year-old Chinese man with nodules of his palms and soles (2). A 62-year-old woman presented with a 14-year history of palmoplantar keratoderma accompanied by a 10-year history of pruritic, lichenoid nodules on the dorsal aspects of her hands and feet. A biopsy of the keratoderma was characteristic of EED (3).

Vesiculobullous forms of EED have been reported, which may need to be distinguished from dermatitis herpetiformis. Rarely, as in the case of a 16-year-old boy with nodules and vesicles on the dorsal hands, direct immunofluorescence may be positive for IgA at the dermoepidermal junction. (4) EED may present with pustules atop more characteristic nodules. Histologically, these demonstrate intraepidermal microabscesses, as in the case of a 49-year-old Chinese man, who responded to dapsone (5).

Occasionally, EED may present with oral ulcerations. Kentley et al reported the case of a 60-year-old Vietnamese man with a 10 year history of banal EED who was also being treated by his otolaryngologist for oral ulcerations that led to the loss of his uvula. (6)

Late EED may easily be clinically mistaken for acral (feet) fibroblastic tumors such as dermatofibrosarcoma protuberans (DFSP) or superficial nodular fasciitis. Such lesions demonstrate a “plywood” appearance characteristic of sclerotic fibromas usually noted in Cowden syndrome, Careful histologic examination demonstrates leukocytoclasia from vasculitis that can secure the diagnosis of EED. Stains for CD34 and S100 are negative, ruling out DFSP or neural neoplasms, respectively. This variant of EED has been reported in a 57-year-old woman with an unremarkable medical history (7) and a 60-year-old African-American, HIV-positive woman. (8) Only knowledge of the existence of this variant of EED combined with a careful search for neutrophilic karyorrhexis would secure the diagnosis.

I always force myself to consider the diagnosis of EED, even with characteristic acral lesions. Paradoxically, with all these variants, diagnosing EED just became easier. Include the diagnosis on virtually any acral inflammatory lesion, and you might be right!

1. Newburger J, Schmieder GJ. Erythema elevatum diutinum. StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2017 Jun- 2017 Oct 16.
2. Keyal U, et al. Erythema elevatum diutinum involving palms and soles: A case report and literature review. Am J Trans Res 2017; 9: 1956-9.
3. Kamyab K, et al. Erythema elevatum diutinum presenting with palmoplantar keratoderma. Clin Exp Dermatol 2017; 42: 573-5.
4. Ossorio-García L. Vesiculobullous variant of erythema elevatum diutinum. Clin Exp Dermatol 2017; 42: 777-80.
5. Ba W, et al. Erythema elevatum diutinum with pustule formation. J Cutan Pathol 2018; Jan 2 [Epub ahead of print].
6. Kentley J, et al. Erythema elevatum diutinum – associated with loss of the uvula. JAAD Case Rep 2017; 3: 212-4.
7. Llamas-Velasco M, et al. Late-stage erythema elevatum diutinum mimicking a fibroblastic tumor: A potential pitfall. Am J Dermatopathol 2017; Dec 28 [Epub ahead of print]
8. Shi KY, Vandergiff T. Late-stage nodular erythema elevatum diutinum mimicking sclerotic fibroma. J Cutan Pathol 2018; 45:94-6.

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