History

Patients with erythema elevatum diutinum (EED) usually present with persistent, firm lesions on the extensor surfaces of their skin, especially over the joints. These lesions are most often nodules and round or oval plaques (see image below). However, on rare occasions, blisters and ulcers may also appear.

Nodular lesions on the knees of a patient with erythema elevatum diutinum.

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The color of the lesions progresses over time from yellow or pinkish to red, purple, or brown. In addition to the color changes, patients may describe the lesions as increasing in number and size over time. They may also note that the lesions enlarge during the day and return to their previous size overnight.^[16]

The lesions can be completely asymptomatic, painful, or cause a sensation of burning or itching. These symptoms can be exacerbated by cold.

The general health of the patient may be otherwise excellent, although a history of arthralgia is found relatively often with erythema elevatum diutinum.

Physical Examination

Upon physical examination, erythema elevatum diutinum lesions are generally found symmetrically on the extensor surfaces of the skin, especially over the joints.^[17]

Clinical studies show a preference for the extensor surfaces of the hands, the wrists, the elbows, the ankles, the Achilles tendons, the fingers, and the toes (see image below).

Plaques and papular lesions on the wrists and the dorsum of the hands and the digits of a patient with erythema elevatum diutinum.

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The buttocks, the face, and the ears as well as the palms, the soles, the legs, the forearms, and the genitals may be involved^[18]; however, the trunk is usually spared.

The lesions usually feel firm and are freely movable over the underlying tissue, except when on the palms and the soles.^[18]

Their surface is generally smooth and sometimes slightly scaly.

Crusting and/or bleeding, although uncommon, have been noted.

Several studies have shown an association of erythema elevatum diutinum with ocular abnormalities, including nodular scleritis, panuveitis, autoimmune keratolysis, and peripheral keratitis.^{[19, 20]}Therefore, attention should be given to ocular symptoms and eye examination findings.

Differential Diagnoses

Sardiña LA, Jour G, Piliang MP, Bergfeld WF. Erythema elevatum diutinum a rare and poorly understood cutaneous vasculitis: A single institution experience. J Cutan Pathol. 2019 Feb. 46 (2):97-101. [QxMD MEDLINE Link].
Hutchinson J. On two remarkable cases of symmetrical purple congestion of the skin in patches, with induration. Br J Dermatol. 1880. 1:10.
Bury JS. A case of erythema with remarkable nodular thickening and induration of the skin associated with intermittent albuminuria. Illus Med News. 1889. 3:145.
Radcliffe-Crocker H, Williams C. Erythema elevatum diutinum. Br J Dermatol. 1894. 6:1-9.
Gibson LE, Su WP. Cutaneous vasculitis. Rheum Dis Clin North Am. 1990 May. 16(2):309-24. [QxMD MEDLINE Link].
Shimizu S, Nakamura Y, Togawa Y, Kamada N, Kambe N, Matsue H. Erythema elevatum diutinum with primary Sjögren syndrome associated with IgA antineutrophil cytoplasmic antibody. Br J Dermatol. 2008 Sep. 159(3):733-5. [QxMD MEDLINE Link].
Grabbe J, Haas N, Moller A, Henz BM. Erythema elevatum diutinum--evidence for disease-dependent leucocyte alterations and response to dapsone. Br J Dermatol. 2000 Aug. 143(2):415-20. [QxMD MEDLINE Link].
Farley-Loftus R, Dadlani C, Wang N, et al. Erythema elevatum diutinum. Dermatol Online J. 2008 Oct 15. 14(10):13. [QxMD MEDLINE Link].
Rover PA, Bittencourt C, Discacciati MP, Zaniboni MC, Arruda LH, Cintra ML. Erythema elevatum diutinum as a first clinical manifestation for diagnosing HIV infection: case history. Sao Paulo Med J. 2005 Jul 7. 123(4):201-3. [QxMD MEDLINE Link].
Futei Y, Konohana I. A case of erythema elevatum diutinum associated with B-cell lymphoma: a rare distribution involving palms, soles and nails. Br J Dermatol. 2000 Jan. 142(1):116-9. [QxMD MEDLINE Link].
Hancox JG, Wallace CA, Sangueza OP, Graham GF. Erythema elevatum diutinum associated with lupus panniculitis in a patient with discoid lesions of chronic cutaneous lupus erythematosus. J Am Acad Dermatol. 2004 Apr. 50(4):652-3. [QxMD MEDLINE Link].
Mitamura Y, Fujiwara O, Miyanishi K, Sato H, Saga K, Ohtsuka K. Nodular scleritis and panuveitis with erythema elevatum diutinum. Am J Ophthalmol. 2004 Feb. 137(2):368-70. [QxMD MEDLINE Link].
Liu TC, Chen IS, Lin TK, Lee JY, Kirn D, Tsao CJ. Erythema elevatum diutinum as a paraneoplastic syndrome in a patient with pulmonary lymphoepithelioma-like carcinoma. Lung Cancer. 2009 Jan. 63(1):151-3. [QxMD MEDLINE Link].
Gubinelli E, Cocuroccia B, Fazio M, Annessi G, Girolomoni G. Papular neutrophilic dermatosis and erythema elevatum diutinum following erythropoietin therapy in a patient with myelodysplastic syndrome. Acta Derm Venereol. 2003. 83(5):358-61. [QxMD MEDLINE Link].
Yiannias JA, el-Azhary RA, Gibson LE. Erythema elevatum diutinum: a clinical and histopathologic study of 13 patients. J Am Acad Dermatol. 1992 Jan. 26(1):38-44. [QxMD MEDLINE Link].
Golmia A, Grinblat B, Finger E, Klieman C, Assir F, Scheinberg M. The development of erythema elevatum diutinum in a patient with juvenile idiopathic arthritis under treatment with abatacept. Clin Rheumatol. 2008 Jan. 27(1):105-6. [QxMD MEDLINE Link].
Di Giacomo TB, Marinho RT, Nico MM. Erythema elevatum diutinum presenting with a giant annular pattern. Int J Dermatol. 2009 Mar. 48(3):290-2. [QxMD MEDLINE Link].
Barzegar M, Davatchi CC, Akhyani M, Nikoo A, Daneshpazhooh M, Farsinejad K. An atypical presentation of erythema elevatum diutinum involving palms and soles. Int J Dermatol. 2009 Jan. 48(1):73-5. [QxMD MEDLINE Link].
Aldave AJ, Shih JL, Jovkar S, McLeod SD. Peripheral keratitis associated with erythema elevatum diutinum. Am J Ophthalmol. 2003 Mar. 135(3):389-90. [QxMD MEDLINE Link].
Casanova FH, Meirelles RL, Tojar M, Martins MC, Rigueiro MP, de Freitas D. Autoimmune keratolysis in a patient with leukocytoclastic vasculitis: unusual erythema elevatum diutinum with granulomatous pattern. Cornea. 2001 Apr. 20(3):329-32. [QxMD MEDLINE Link].
Ayoub N, Charuel JL, Diemert MC, et al. Antineutrophil cytoplasmic antibodies of IgA class in neutrophilic dermatoses with emphasis on erythema elevatum diutinum. Arch Dermatol. 2004 Aug. 140(8):931-6. [QxMD MEDLINE Link].
Weidman FD, Besancon JH. Erythema elevatum diutinum: role of streptococci, and relationship to other rheumatic dermatoses. Arch Dermatol Syphilol. 1929. 20:593.
Wolff HH, Maciejewski W, Scherer R. [Erythema elevatum diutinum. I. Electron microscopy of a case with extracellular cholesterosis (author's transl)]. Arch Dermatol Res. 1978 Feb 15. 261(1):7-16. [QxMD MEDLINE Link].
Katz SI, Gallin JI, Hertz KC, Fauci AS, Lawley TJ. Erythema elevatum diutinum: skin and systemic manifestations, immunologic studies, and successful treatment with dapsone. Medicine (Baltimore). 1977 Sep. 56(5):443-55. [QxMD MEDLINE Link].
Kohler IK, Lorincz AL. Erythema elevatum diutinum treated with niacinamide and tetracycline. Arch Dermatol. 1980 Jun. 116(6):693-5. [QxMD MEDLINE Link].
Chow RK, Benny WB, Coupe RL, Dodd WA, Ongley RC. Erythema elevatum diutinum associated with IgA paraproteinemia successfully controlled with intermittent plasma exchange. Arch Dermatol. 1996 Nov. 132(11):1360-4. [QxMD MEDLINE Link].
Ahmad S, Delarosa M, Kleinman W, Ahmad R. Primary Surgical Treatment of Erythema Elevatum Diutinum. J Hand Surg Am. 2018 Sep 18. [QxMD MEDLINE Link].
Tasanen K, Raudasoja R, Kallioinen M, Ranki A. Erythema elevatum diutinum in association with coeliac disease. Br J Dermatol. 1997 Apr. 136(4):624-7. [QxMD MEDLINE Link].

Media Gallery

Plaques and papular lesions on the wrists and the dorsum of the hands and the digits of a patient with erythema elevatum diutinum.
Nodular lesions on the knees of a patient with erythema elevatum diutinum.
Fibrinoid changes in dermal blood vessels with polymorphonuclear neutrophil infiltration.

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Author

Firas G Hougeir, MD Staff Dermatologist, Private Practice, Georgia

Firas G Hougeir, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

James A Yiannias, MD Associate Professor of Dermatology, Associate Dean, Mayo School of Graduate Medical Education, Mayo Foundation for Medical Education and Research; Vice Chair, Medical Division, Department of Dermatology, Mayo Clinic Scottsdale

James A Yiannias, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Christen M Mowad, MD Professor, Department of Dermatology, Geisinger Medical Center

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, Noah Worcester Dermatological Society, Pennsylvania Academy of Dermatology, American Academy of Dermatology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Carrie L Kovarik, MD Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Erythema Elevatum Diutinum Clinical Presentation

History

Physical Examination

References

Tables

Contributor Information and Disclosures

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