Erythema elevatum diutinum: a case report and review of literature

Abstract

Erythema elevatum diutinum (EED) is a rare cutaneous leukocytoclastic vasculitis thought to be related to increased levels of circulating antibodies. It has been shown to be associated with HIV infection, tuberculosis, as well as various autoimmune diseases. A retrospective review of all cases of EED indexed in PubMed between 1990 and 2014 was performed. Inclusion criteria for articles was availability of full text in English and a biopsy-confirmed diagnosis of EED. All other articles were excluded. Cases were stratified by age and anatomic location of the lesions. Treatment response was coded as "complete," "partial," and "none." A total of 133 cases of EED with 381 lesions detailed in case reports and case series were included. Twenty-one cases were associated with HIV. Of 47 patients with reported paraproteinemias, IgA paraproteinemia was found in 57.45%, IgG paraproteinemia in 29.8%, IgM paraproteinemia in 10.6%, and IgD paraproteinemia in 2.1% of cases. Of 40 (30.1%) patients with reported comorbid autoimmune disease, rheumatoid arthritis was associated with 10 cases. Cancer was found to be associated with 9.77% of cases. Seventy-five patients were treated with dapsone, with 36 (48%) achieving complete treatment response, 24 (32%) achieving partial response, and seven (9.3%) achieving no response. Keeping the clinical associations of EED in mind, especially malignancy, is critical in management of the disease. More structured studies need to take place in order to fully define the mechanisms and strength of these associations.

Concurrence of erythema elevatum diutinum and HIV infection: A case report and literature review

“…A male sex predominance was noted with 81.8% (27 of 33) of patients in Table I. Men make up 55.6% of all cases of EED 2 ; however, the findings of this review are consistent with prior findings of HIV-associated EED 1, 2, 3. This finding may reflect male/female population disparities with HIV as opposed to a true tendency toward men with HIV.…”

“…EED is a rare chronic cutaneous small-vessel leukocytoclastic vasculitis that presents initially as soft, nonfixed red/violaceous or brown papules and nodules that later become firm because of fibrosis. The lesions are distributed most frequently on extensor surfaces of the hands, arms, and legs 1 , 2 . Involvement of the feet and plantar surface (as with our patient) is considered unusual and may represent as few as 4.2% of EED cases 2 .…”

“…The lesions are distributed most frequently on extensor surfaces of the hands, arms, and legs 1 , 2 . Involvement of the feet and plantar surface (as with our patient) is considered unusual and may represent as few as 4.2% of EED cases 2 . Its presentation may mimic that of Kaposi sarcoma, bacillary angiomatosis, granuloma annulare, and Sweet syndrome among others, and as such, histologic correlation is necessary for diagnosis 1 .…”

“…As lesions progress to late stage, there is greater involvement of histiocytes and dermal spindle cells leading to fibrosis of lesions that may resemble sclerosing hemangioma 1 . The pathophysiology of this condition is likely caused by immune complex deposition in small vessels leading to an inflammatory cascade 1 , 2 …”

“…In their review of the 86 cases published between 1977 and 2012, Momen et al 1 found EED to be associated with many other disease processes including HIV (n = 19/86 [22%]), IgA gammopathy, bacterial infection, and inflammatory bowel disease among others 1 . More recently, Doktor et al, 2 in their 2019 review of 133 cases of EED between 1990 and 2014, found 21 cases associated with HIV. Here we review the above case as well as the 32 electronically indexed cases of HIV-associated EED with reported CD4 counts that were published between 1986 and 2018.…”