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Erythema Elevatum Diutinum

  • Author: Firas G Hougeir, MD; Chief Editor: William D James, MD  more...
 
Updated: Jun 03, 2016
 

Background

Erythema elevatum diutinum (EED) is a rare type of leukocytoclastic vasculitis characterized by red, purple, brown, or yellow papules, plaques, or nodules. These lesions are usually distributed on the extensor surfaces of the body. Erythema elevatum diutinum was first described in 1888 by Hutchinson[1] and in 1889 by Bury.[2] However, the name erythema elevatum diutinum was first used by Radcliff-Crocker and Williams[3] who found similarities between the cases of Hutchinson and Bury and their own. No clinical examination difference is apparent between the Hutchinson type and the Bury type of erythema elevatum diutinum. The difference lies in the patient population and possibly the cause of onset.

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Pathophysiology

The pathophysiology of erythema elevatum diutinum is not well understood. According to Gibson and Su,[4] the lesions are thought to be caused by the deposition of immune complexes in small blood vessels. This induces an inflammatory cascade, which damages the vessels. This repetitive damage causes fibrosis and the appearance of cholesterol crystals and myelin figures in the vessels. Direct immunofluorescence shows deposits of complement as well as immunoglobulin G (IgG), immunoglobulin (IgM), immunoglobulin A (IgA),[5] and fibrin around the damaged vessels. The findings from Grabbe et al[6] support the idea that the initiation of erythema elevatum diutinum may occur via activation of cytokines such as interleukin 8, which causes selective recruitment of leukocytes to tissue sites.[7]

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Epidemiology

Frequency

Erythema elevatum diutinum is a rare disease. Although first described in 1888, the largest study of erythema elevatum diutinum was published in 1992 and included 13 patients.[8]

Race

No racial predilection is reported for erythema elevatum diutinum.

Sex

Erythema elevatum diutinum is found in both males and females. The Hutchinson type of erythema elevatum diutinum is predominant in men. The Bury type of erythema elevatum diutinum is found in women with a history of rheumatologic disease.

Age

Erythema elevatum diutinum can occur at any age. However, erythema elevatum diutinum is mostly an adult disease that occurs from the third to sixth decade of life. Men with erythema elevatum diutinum are usually older (Hutchinson type), and women are usually younger (Bury type).

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Prognosis

Erythema elevatum diutinum is a chronic disease that usually evolves over a 5- to 10-year period, at which point it may resolve. Erythema elevatum diutinum lesions tend to not leave scars, but areas of hyperpigmentation or hypopigmentation can be visible.

No mortality due to erythema elevatum diutinum has been reported. Erythema elevatum diutinum lesions can be completely asymptomatic, but they may be cosmetically disturbing. In other cases, the lesions can be associated with pain, itching, and/or a burning sensation. The most common systemic symptom is joint pain.

Dapsone and other therapies can be successful in limiting the progression of the disease.

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Contributor Information and Disclosures
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: Nothing to disclose.

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.

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. 1990 May. 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. 2008 Sep. 159(3):733-5. [Medline].

  6. 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. [Medline].

  7. Farley-Loftus R, Dadlani C, Wang N, et al. Erythema elevatum diutinum. Dermatol Online J. 2008 Oct 15. 14(10):13. [Medline].

  8. 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. [Medline].

  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. 2008 Jan. 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. 2009 Mar. 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. 2009 Jan. 48(1):73-5. [Medline].

  12. Aldave AJ, Shih JL, Jovkar S, McLeod SD. Peripheral keratitis associated with erythema elevatum diutinum. Am J Ophthalmol. 2003 Mar. 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. 2001 Apr. 20(3):329-32. [Medline].

  14. 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. [Medline].

  15. 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. [Medline].

  16. 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. [Medline].

  17. 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. [Medline].

  18. 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. [Medline].

  19. 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].

  20. 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. [Medline].

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

  22. 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. [Medline].

  23. 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. [Medline].

  24. Kohler IK, Lorincz AL. Erythema elevatum diutinum treated with niacinamide and tetracycline. Arch Dermatol. 1980 Jun. 116(6):693-5. [Medline].

  25. 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. [Medline].

  26. 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. [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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