Erythema elevatum diutinum a rare and poorly understood cutaneous vasculitis: A single institution experience

Abstract

Background Erythema elevatum diutinum (EED) is a rare vasculitis with variable clinical presentation which diagnosis can be challenging. Herein we want to describe the clinicopathological spectrum of findings in five cases of EED. Methods We retrospectively analyzed five cases in a single institution collected over a period of 27 years. The clinical history was collected and all the slides were examined to determine the histopathological characteristics of the lesions. Results The mean age of our patients is 56.6 years. Two of five patients were females and with lesions showing predilection for the upper‐extremities. The most common presentation was of an erythematous plaque on the extensor surfaces. Three patients had a history of neoplasm. All cases showed leukocytoclastic damage consistent with EED. Conclusion Our findings suggest that EED shows a heterogeneous clinical and pathological presentation, which can show an overlap with granulomatous dermatoses and mixed connective tissue diseases. Scalp lesions can occur and can mimic granulomatous dermatoses. The finding of EED in benign and malignant solid tumors in three of our patients begs the question whether there is an association between EED and such solid neoplasms.

Atypical Presentation of Erythema Elevatum Diutinum in a Patient With Hashimoto's Disease

“…The classic presentation of EED involves most commonly the extensor surfaces of the arms and legs and less commonly the trunk, head, and neck. Atypical presentations of EED have been rarely noted on the palms/soles, genitals, and oral cavity [1,3]. To our knowledge, the appearance of EED lesions under dermoscopy is not well-described in the literature.…”

“…One theory attributes its development to fibrosis from inflammatory cytokines, specifically interleukin-8 [4]. Other theories include a type III hypersensitivity reaction leading to vasculopathy [3]. Histopathologic findings correspond to the age of the lesion: newer onset lesions show neutrophilic infiltrate, while chronic lesions show fibrosis of vessel walls and leukocytoclasia [2].…”

“…These lesions may be asymptomatic but can also be painful and distressing to patients. Diagnosis is established by biopsy and histopathologic findings of leukocytoclasia and vessel fibrosis [2,3]. Subsequent laboratory examinations are often conducted for associated conditions.…”

Violaceous nodules over dorsal interphalangeal joints in a patient with HIV

“…Erythema elevatum diutinum is a rare, chronic dermatosis characterized by red-violet papules or nodules over extensor surfaces. An earlier onset occurs more often in the setting of HIV infection 4 . Histopathologic features consist of a fibrosing leukocytoclastic vasculitis with a neutrophilic infiltrate.Granuloma annulare – Incorrect.…”

“…An earlier onset occurs more often in the setting of HIV infection. 4 Histopathologic features consist of a fibrosing leukocytoclastic vasculitis with a neutrophilic infiltrate.…”