Erythema Elevatum Diutinum Clinical Presentation

  • Author: Firas G Hougeir, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jun 3, 2010
 

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

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.[9]

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.

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Physical

Upon physical examination, erythema elevatum diutinum lesions are generally found symmetrically on the extensor surfaces of the skin, especially over the joints.[10]

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

The buttocks, the face, and the ears as well as the palms, the soles, the legs, the forearms, and the genitals may be involved[11] ; 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.[11]

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.[12, 13] Therefore, attention should be given to ocular symptoms and eye examination findings.

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Causes

The cause of erythema elevatum diutinum (EED) has not yet been definitively established.

  • Disorders that have been associated with erythema elevatum diutinum include recurrent bacterial infections (especially streptococcal), viral infections (including hepatitis B or HIV), rheumatologic disease (in the Bury type), lupus erythematosus, and B-cell lymphoma.[14, 15, 16, 17]
  • In recent years, several reports, including the 2 largest clinical studies completed on erythema elevatum diutinum, have suggested hematologic disease as the most common factor associated with erythema elevatum diutinum.
  • Monoclonal gammopathies, especially IgA monoclonal gammopathy, have been found in a significant number of patients in several studies.
  • Erythema elevatum diutinum has also been reported after the administration of erythropoietin.[18]
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Contributor Information and Disclosures
Author

Firas G Hougeir, MD  Staff Physician, Department of Dermatology, Mayo Clinic Scottsdale

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

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.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

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

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Hutchinson J. On two remarkable cases of symmetrical purple congestion of the skin in patches, with induration. Br J Dermatol. 1880;1:10.

  2. 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.

  3. Radcliffe-Crocker H, Williams C. Erythema elevatum diutinum. Br J Dermatol. 1894;6:1-9.

  4. Gibson LE, Su WP. Cutaneous vasculitis. Rheum Dis Clin North Am. May 1990;16(2):309-24. [Medline].

  5. 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. Sep 2008;159(3):733-5. [Medline].

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  9. 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. Jan 2008;27(1):105-6. [Medline].

  10. Di Giacomo TB, Marinho RT, Nico MM. Erythema elevatum diutinum presenting with a giant annular pattern. Int J Dermatol. Mar 2009;48(3):290-2. [Medline].

  11. 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. Jan 2009;48(1):73-5. [Medline].

  12. Aldave AJ, Shih JL, Jovkar S, McLeod SD. Peripheral keratitis associated with erythema elevatum diutinum. Am J Ophthalmol. Mar 2003;135(3):389-90. [Medline].

  13. 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. Apr 2001;20(3):329-32. [Medline].

  14. 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. Jan 2000;142(1):116-9. [Medline].

  15. 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. Apr 2004;50(4):652-3. [Medline].

  16. Mitamura Y, Fujiwara O, Miyanishi K, Sato H, Saga K, Ohtsuka K. Nodular scleritis and panuveitis with erythema elevatum diutinum. Am J Ophthalmol. Feb 2004;137(2):368-70. [Medline].

  17. 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. Jan 2009;63(1):151-3. [Medline].

  18. 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. [Medline].

  19. 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. Aug 2004;140(8):931-6. [Medline].

  20. Weidman FD, Besancon JH. Erythema elevatum diutinum: role of streptococci, and relationship to other rheumatic dermatoses. Arch Dermatol Syphilol. 1929;20:593.

  21. Wolff HH, Maciejewski W, Scherer R. [Erythema elevatum diutinum. I. Electron microscopy of a case with extracellular cholesterosis (author's transl)]. Arch Dermatol Res. Feb 15 1978;261(1):7-16. [Medline].

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  25. Tasanen K, Raudasoja R, Kallioinen M, Ranki A. Erythema elevatum diutinum in association with coeliac disease. Br J Dermatol. Apr 1997;136(4):624-7. [Medline].

  26. Devillierre M, Verola O, Rybojad M, et al. [Pseudoneoplastic lesion of erythema elevatum diutinum]. Ann Dermatol Venereol. Aug-Sep 2008;135(8-9):575-9. [Medline].

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  28. Habif TP. Clinical Dermatology. 3rd ed. St. Louis, Mo: Mosby-Year Book; 1996:589-96.

  29. Hines HL. Erythema elevatum diutinum. Dermatol Int. Apr-Jun 1968;7(2):70-4. [Medline].

  30. Laymon CW. Erythema elevatum diutinum. A type of allergic vasulitis. Arch Dermatol. Jan 1962;85:22-8. [Medline].

  31. McEvoy GK, ed. AHFS Drug Information, 1999. Bethesda, Md: American Society of Health-System Pharmacists; 1999:731-5, 3182-4.

  32. Mraz JP, Newcomer VD. Erythema elevatum diutinum. Presentation of a case and evaluation of laboratory and immunological status. Arch Dermatol. Sep 1967;96(3):235-46. [Medline].

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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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