Share on Facebook
Share on Twitter
Share on LinkedIn
Share by Email
Print
Slideshow
-
Slide
A 40-year-old woman presents to the clinic with multiple red-brown and violaceous nodules along the extensor surface of her fingers that have recurred over the past 3 years. She states the lesions “come and go.” The patient reports that the lesions are soft and light in color initially then become firmer and darker with time. She denies any pain, irritation, or burning sensation associated with the nodules. Medical history is significant for HIV infection and inflammatory bowel disease.
The patient is diagnosed with erythema elevatum diutinum (EED), a rare chronic dermatosis. Erythema elevatum diutinum typically presents as cutaneous lesions of red-brown, violaceous, or yellowish papules, plaques, or nodules favoring the extensor surfaces and acral sites.1 Lesions usually begin as...
Submit your diagnosis to see full explanation.
The patient is diagnosed with erythema elevatum diutinum (EED), a rare chronic dermatosis. Erythema elevatum diutinum typically presents as cutaneous lesions of red-brown, violaceous, or yellowish papules, plaques, or nodules favoring the extensor surfaces and acral sites.1 Lesions usually begin as erythematous, soft lesions and become red-brown, violaceous, or indurated over time.1
Histologically, early lesions show leukocytoclastic vasculitis (LCV), while later lesions exhibit LCV with mixed infiltrate (eosinophils, lymphocytes, neutrophils, and plasma cells), including histiocytes (often seen in chronic inflammatory processes), as well as foci of storiform or concentric ribbons of collagen (onion-skin fibrosis).1,2 In late lesions, the storiform or concentric ribbons of collagen predominate and LCV changes become less prominent.2
Erythema elevatum diutinum lesions are often asymptomatic, with some patients experiencing pain or a burning sensation.3 This skin condition commonly occurs in patients 30 to 70 years of age, but patients can develop this disease at any age without any sex or racial preference.3 The condition has been associated with extracutaneous findings such as arthralgias and scleritis, and is typically associated with systemic diseases including HIV, immunoglobulin A (IgA) paraproteinemia, myelomas, neutrophilic dermatoses, and inflammatory bowel disease.1 Dapsone is currently the treatment of choice, with the majority of patients achieving a complete or partial response.1,4 Other treatments consist of anti-inflammatory, antimicrobial, and immunosuppressive agents.1 For HIV-positive patients, antiretroviral therapy is also prescribed if the patient is not currently prescribed this treatment.1
The chronic and recurrent nature of EED is the primary means of distinguishing it from other entities.5 The differential diagnosis includes granuloma faciale, Sweet syndrome (acute febrile neutrophilic dermatosis), dermatofibroma, and Kaposi sarcoma, which can be distinguished from EED by the appearance of vasculitis involving the face, fever, nonfluctuating tumor mass, or excessive proliferation of spindle cells, respectively.6,7
The patient in this case was started on dapsone and the lesions respond well to treatment.
Xinxin Wu, BS, BA, is a medical student at Creighton University School of Medicine; Annie Jin, MD, is a resident physician in the Department of Dermatology at Temple University Lewis Katz School of Medicine.
References
1. Sandhu JK, Albrecht J, Agnihotri G, Tsoukas MM. Erythema elevatum et diutinum as a systemic disease. Clin Dermatol. 2019;37(6):679-683. doi:10.1016/j.clindermatol.2019.07.028
2. LeBoit PE, Yen TS, Wintroub B. The evolution of lesions in erythema elevatum diutinum. Am J Dermatopathol. 1986;8(5):392-402. doi:10.1097/00000372-198610000-00005
3. Newburger J, Schmieder GJ. Erythema elevatum diutinum. In: StatPearls [Internet]. StatPearls Publishing; September 5, 2022. https://www.ncbi.nlm.nih.gov/books/NBK448069/.
4. Yiannias JA, el-Azhary RA, Gibson LE. Erythema elevatum diutinum: a clinical and histopathologic study of 13 patients. J Am Acad Dermatol. 1992;26(1):38-44. doi:10.1016/0190-9622(92)70003-x
5. Wahl CE, Bouldin MB, Gibson LE. Erythema elevatum diutinum: clinical, histopathologic, and immunohistochemical characteristics of six patients. Am J Dermatopathol. 2005;27(5):397-400. doi:10.1097/01.dad.0000175526.89249.be
6. Bolognia JL, Schaffer JV, Cerroni L. Dermatology, 4th edition. Elsevier; 2017. 7. Rao GR, Joshi R, Phaneendra Prasad AK, Amareswar A, Sandhya S, Sridevi M. Nodular erythema elevatum diutinum mimicking Kaposi’s sarcoma in a human immunodeficiency virus infected patient. Indian J Dermatol. 2014;59(6):592-4. doi:10.4103/0019-5154.143527
QUESTION
