Erythema elevatum diutinum in acquired immune deficiency syndrome: Can it be an immune reconstitution inflammatory syndrome?

Abstract

A 47-year-old male with acquired immune deficiency syndrome (AIDS) presented with multiple hyperpigmented papules and nodules on both ankles, dorsum of bilateral feet and soles. It was associated with mild itching and pain. The patient was diagnosed with human immunodeficiency virus (HIV) in 2007. First-line antiretroviral therapy (ART) was started in 2009 to which he responded initially. He was shifted to second-line ART 11 months ago in March 2015 due to treatment failure as suggested by CD4 count of 50 cells/mm3. The present skin lesions started 2 months after the initiation of second-line ART. Differential diagnoses considered were Kaposi's sarcoma and immune reconstitution inflammatory syndrome (IRIS) related infections, but biopsy was suggestive of erythema elevatum diutinum (EED). Patient was started on oral dapsone 100 mg/day and increased to 200 mg/day to which he is responding gradually. In the present case, appearance of the lesions after initiation of second-line ART coupled with increase in CD4 count and decrease of viral load below undetectable level suggest that EED could be an IRIS.

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

“…Surgical excision may be appropriate in the setting of late-stage, fibrotic lesions 19 . However, of the 10 late-stage lesions treated with dapsone with known response, 5 completely or partially resolved, 3 discontinued dapsone because of adverse events, and 2 did not respond to treatment 3 , 5, 6, 7 , 9 , 10 , 14 , 16 , 19 , 20 . This finding may indicate that surgical excision (or other therapy) is more appropriate after treatment failure with dapsone.…”

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

“…The morphological appearance of these lesions correlates well with the repair/fibrosis that follows the initial inflammatory response . It is believed that the initiation of EED occurs via activation of cytokines, especially interleukin 8 (IL‐8) . The hallmark histological features in early lesions are those of a leukocytoclastic vasculitis involving the papillary dermis and mid‐dermal vessels resulting in a dense neutrophilic infiltrate with fibrinoid necrosis.…”

“…Within the viral infections, HIV infection has been reported as one of the most common associated causes of EED; even being considered initially an emerging pathognomonic HIV‐related dermatosis . EED has been associated also to immune reconstitution inflammatory syndrome, seen in HIV patients . Disorders, such as Kaposi sarcoma, sarcoidosis, and pseudo‐lymphoma have been associated to EED …”

“…4 It is believed that the initiation of EED occurs via activation of cytokines, especially interleukin 8 (IL-8). 4,5 The hallmark histological features in early lesions are those of a leukocytoclastic vasculitis involving the papillary dermis and middermal vessels resulting in a dense neutrophilic infiltrate with fibrinoid necrosis. As the lesions age, the most common hallmark histology feature is an onion skin-like fibrosis surrounding the vessels.…”

“…9 EED has been associated also to immune reconstitution inflammatory syndrome, seen in HIV patients. 5 Disorders, such as Kaposi sarcoma, sarcoidosis, and pseudo-lymphoma have been associated to EED. 9 For inexperienced dermatopathologists, the diagnosis of EED can be challenging, especially because the inflammatory damage is not exclusively specific to EED, and it may vary as the lesions age.…”