Abstract
Kaposi’s sarcoma (KS) is the commonest human immunodeficiency virus (HIV)-related malignancy with its characteristic cutaneous morphological appearance and histopathological features. However, it can be simulated by other co-morbid opportunistic infections and unrelated dermatological conditions. We describe such a case of acroangiodermatitis in an HIV co-infected man, based on exclusion of KS histologically and the absence of human herpesvirus 8, the causative agent of KS.
Introduction
Skin manifestations are common in human immunodeficiency virus (HIV) patients and in addition to infective, proliferative and drug disorders also include neoplastic lesions, the commonest of which is Kaposi’s sarcoma (KS). Cutaneous KS is diagnosed on the clinical characteristic morphology of skin lesions often without histological confirmation or evidence of human herpesvirus 8 (HHV-8) identification, the causal agent. Whilst occurring more commonly with late or advanced HIV disease, KS does still occurs in patients with CD4 cell counts above 500 cells/mm³. 1 We describe a case where clinical appearance was highly suggestive of KS, but an alternative diagnosis was considered and confirmed by histology and the absence of HHV-8 immunohistochemistry and serology.
Case report
A 42-year-old Caucasian man who has sex with men was diagnosed with HIV in 2005 but disengaged from care until 2012 when he subsequently resumed his HIV monitoring and care. He reported to be well and was clinically asymptomatic. His CD4 cell count was 310 cells/mm³ and HIV viral load 3946 copies/ml with negative syphilis and hepatitis C serology and was hepatitis B core antibody positive, surface antigen negative. He was commenced on tenofovir/emtricitabine/rilpiverine one tablet daily and subsequently became fully virologically suppressed with an HIV viral load less than 40 copies/ml, and with CD4 cell counts consistently greater than 500 cells/mm³. He remained well until 2015, when he started to complain of a painful purplish eruption on both his feet and ankles and later spreading to his shins. They were described as scaly but developing into painful ulcers which precluded him wearing normal footwear. On examination, he had marked purplish ulcerative plaque-type lesions with a reticular pattern on his shins and gaitor area (Figure 1), dorsum of feet and posterior ankles, with areas of hyperpigmentation (Figures 2 and 3). No pedal oedema or inguinal lymphadenopathy was present, and his peripheral pulses were present. It was also noted that he had an acneiform eruption on his trunk consistent with comedonal acne.
Bilateral pigmented lesions with reticular pattern on shins and feet.
Ulcerative plaque on ankle with surrounding hyperpigmentation.
Scaly ulcerative plaques on heels.
Diagnosis and subsequent progress
In addition to KS, the differential diagnosis included either vasculitis or vascular insufficiency and secondary syphilis. A full autoimmune screen including autoantibodies and repeat syphilis serology (Treponemal enzyme immunoassay [EIA], Treponema pallidum haemagglutination assay [TPHA], rapid plasma reagin [RPR]) were all negative. He underwent full vascular assessment, including colour Doppler ultrasound which concluded that there was no evidence of vascular or arterial insufficiency present in the limbs. As it was not possible to exclude a diagnosis of KS based on clinical appearance alone, full dermatological assessment with cutaneous biopsy was made in addition to immunostaining for anti-CD34 and HHV-8 serology which was reported as negative. The histopathological appearance was supportive of the clinical diagnosis of acroangiodermatitis with diffuse dilated vessels and capillaries (Figure 4) and the classical features of spindle cell proliferation with slit-like spaces, the hallmark of KS, were absent. Despite the overlap with KS both clinically and histologically, the absence of HHV-8 DNA in samples and sera favoured acroangiodermatitis as the correct diagnosis. Despite it being a benign condition symptomatic treatment with compression stocking, topical corticosteroid and emollients did not significantly help, and he subsequently required management at a pain clinic.
H & E stain of skin biopsy showing dilated blood vessels and proliferation of capillaries with haemosiderin deposition within the dermis, in the absence of HHV-8 on immunostaining.
Discussion
Acroangiodermatitis (pseudo-KS) is an uncommon condition felt to be a non-tumoral angioproliferative disorder which resembles KS clinically and morphologically and frequently requires cutaneous biopsy and histopathology to be reliably distinguished. 2 In the early stages, it resembles stasis dermatitis histologically progressing to endothelial cell proliferation and haemosiderin deposition.3,4 The underlying cause is frequently related to chronic venous insufficiency which was present in this case and less commonly with hereditary coagulation disorders such as Bluefarb Syndrome associated with Arteriovenous (AV) malformations and limb amputation.3,4 To our knowledge, there has been only one previously documented case of acroangiodermatitis with HIV co-infection, 5 this in association with advanced HIV disease and hepatitis C. This case highlights the importance of awareness of this condition as a simulator of KS, together with biopsy and the absence of HHV-8 DNA detection in serological and histopathological samples.
Footnotes
Declaration of conflicting interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The authors received no financial support for the research, authorship, and/or publication of this article.