SUBMITTED 11 NOV 21 1 

REVISION REQ. 12 DEC 21; REVISIONS RECD. 26 DEC 21 2 

ACCEPTED 1 FEB 22 3 

ONLINE-FIRST: FEBRUARY 2022 4 

DOI: https://doi.org/10.18295/squmj.2.2022.018 5 

 6 

Dermatological Lesions of Cholesterol Embolization Syndrome and Kaposi 7 

Sarcoma Mimic Primary Systemic Vasculitis 8 

Case Report Study 9 

Abdulmohsen S. Alqurashi,1 Mohammed H. Aly,2 Abdulghaffar Mohammed,2 10 

Walaa A. Ahmed,1 Abdullah M. Alhazmi,1 Amal A. Ahmed,3 Abdulrahman A. 11 

Alshehri,4 *Abdulrahman M. Almalki1 12 

 13 

1College of Medicine, Umm Al-Qura University, Makkah, Saudi Arabia; Departments of 14 

2Internal Medicine, 3Histopathology and 4Rheumatology, Security Forces Hospital, Makkah, 15 

Saudi Arabia. 16 

*Corresponding Author’s e-mail: amalki2023@gmail.com 17 

 18 

Abstract 19 

Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from 20 

systemic non-specific features such as fever, malaise, arthralgia, and myalgia to specific organ 21 

damage. We describe two cases of cholesterol embolization syndrome and Kaposi sarcoma 22 

mimicking primary systemic vasculitis, both of which were characterized by features such as 23 

livedo reticularis, blue toe syndrome, a brown, purpuric skin rash, and positive p-ANCA 24 

associated with Kaposi sarcoma. Establishing the right diagnosis was challenging, and thus 25 

we aim in this study to highlight the possible ways to distinguish them from primary systemic 26 

vasculitis. 27 

Keywords: Dermatological lesions, Cholesterol embolization syndrome, Kaposi sarcoma, 28 

vasculitis mimic  29 

 30 



 

 
 

Introduction 31 

Vasculitis is an inflammatory process affecting the blood vessels, causing destruction that leads 32 

to ischemia, hemorrhage, or both (1). It has a wide spectrum of manifestations ranging from 33 

systemic non-specific features such as fever and a loss of appetite or weight to specific organ 34 

damage such as kidney and/or cutaneous damage manifested by purpura nodules, purpuric 35 

urticaria, livedo reticularis, and skin ulcers (2,3). Thus, non-specific diverse manifestations can 36 

be mistaken for other conditions. We report two cases of the rare presentation of cholesterol 37 

embolization syndrome (CES) and Kaposi sarcoma (KS) mimicking vasculitis. We aim to 38 

prompt their recognition and distinguish them from vasculitis. 39 

 40 

Case presentation 41 

Case One 42 

A 65-year-old man, a known case of triple coronary artery vessel disease based on a coronary 43 

angiogram performed a week before, presented to our hospital with abdominal pain and vomiting 44 

for a day. Physical examination revealed a skin rash, livedo reticularis, and the blue discoloration 45 

of the toes, which suggested blue toe syndrome (Figure 1). All other physical examinations were 46 

unremarkable. 47 

 48 

The patient was admitted, and laboratory investigations showed the following results: white 49 

blood cell count of 14/mm3 with neutrophiles 53%, eosinophile 9%, and lymphocytes 32%; 50 

hemoglobin 11.1 g/dL; platelets 144 × 10³/mm³; erythrocyte sedimentation rate 32 mm/h; C-51 

reactive protein 35 mg/L; creatinine 2.4 mg/dL; and urea 43 mg/dL; aspartate aminotransferase 52 

36 U/L (normal range, 10–41 U/L); alanine aminotransferase 31 U/L (normal range,10–40 U/L); 53 

total cholesterol 209 mg/dL; high-density lipoprotein 42 mg/dL; low-density lipoprotein 138 54 

mg/dL; triglyceride 140 mg/dL; serum iron 64lg/dL (normal range, 50–140 lg/dL); serum ferritin 55 

138 ng/ml (normal range, 15–300 ng/ml); transferrin iron binding capacity 238 lg/dL (normal 56 

range, 130–350 lg/dl). The urinary protein level was 600 mg/dL and the microscopic urinary 57 

examination was negative for cells and casts.  Serological testing for anti-nuclear antibodies, 58 

anti-neutrophil cytoplasmic antibodies, hepatitis C virus, and hepatitis B surface antigen all were 59 

negative.  60 



 

 
 

On abdominal ultrasound, both kidneys were within normal size with no other remarkable 61 

findings. Transthoracic echocardiography showed the left ventricle normal in size with an 62 

ejection fraction of 57%, no intramural thrombus, or any evidence of infective endocarditis. 63 

Multiple biopsies of the affected skin and kidney were arranged but, unfortunately, were refused 64 

by the patient. 65 

 66 

Case Two 67 

An 86-year-old woman presented with intermittent fever, dyspnea, a dry cough, and a loss of 68 

weight for two months. Physical examination revealed a bilateral brown/purple ill-demarcated 69 

plaque over the tibia and dorsum of the feet (Figure 2). Laboratory investigation revealed mild 70 

thrombocytopenia (124×10³/mm³), anemia (10.6 g/dL), high erythrocyte sedimentation rate (55 71 

mm/h), and high C-reactive protein (42 mg/L); serological testing was positive for perinuclear 72 

anti-neutrophil cytoplasmic antibodies (p-ANCA), and enzyme-linked immunoassay testing 73 

revealed positive anti-myeloperoxidase with a titre of 512 AAU/ml (normal: 0-150 AAU/ml), 74 

and negative for anti-proteinase 3. All other investigations were within the normal range 75 

including coagulation profile, renal function, and HBV and HCV testing. Multiple biopsies of 76 

lesions were taken and these showed areas of hemorrhage, a vague network of connecting 77 

channels, and positive human herpesvirus-8 on immunohistostaining (Figure 3), which is 78 

consistent with KS. Human immunodeficiency virus testing was carried out and was negative. 79 

 80 

Declaration of patient consent 81 

The authors certify that they obtained all appropriate patient consent forms. In the form, patients 82 

provided consent for their images and other clinical information to be reported in the journal. 83 

They understand that their names and initials will not be published, and due efforts will be made 84 

to conceal their identity, but anonymity cannot be guaranteed. 85 

 86 

Discussion 87 

Multiple conditions can injure or occlude the blood vessels and mimic the clinical picture of 88 

vasculitis. The location and size of the affected vessel determine the clinical manifestations of 89 

the vascular injury more than the underlying cause. For example, damage to small cutaneous 90 

vessels is manifested by palpable purpura, urticaria, livedo reticularis, papulovesicular lesions, 91 



 

 
 

and nodules. Similarly, damage can arise in the heart, kidney, gastrointestinal tract, and brain (4). 92 

These diseases are not necessarily associated with blood wall inflammation. However, they 93 

might have the same findings of vasculitis in clinical, laboratory, radiographic and/or pathologic 94 

settings, which leads to diagnostic confusion (5). 95 

 96 

CES is a great mimic, which makes it confusing for the diagnosis of vasculitis. A purpuric rash, 97 

livedo reticularis, myalgia, and acute renal failure are some of the symptoms that can occur. 98 

Cholesterol emboli can complicate cardiac catheterization and arteriography; moreover, this can 99 

happen spontaneously, even in individuals who have never suffered previous vascular disease 100 

(6). In our first case, the patient developed manifestations that mimic the typical features of 101 

Churg–Strauss vasculitis such as livedo reticularis, purpura, skin ulceration, and infarction and 102 

eosinophilia (6). 103 
 104 

Moreover, renal failure can be found in both syndromes. While this can mask diagnosis, multiple 105 

biopsies of affected sites (skin, kidney, and muscle) can identify the characteristics of CES, 106 

namely, occluded small arteries and arterioles characterized by a lance-shaped cleft (dissolution 107 

of cholesterol crystals) (7). Unfortunately, our patient refused the biopsy and the diagnosis was 108 

made based on clinical pictures. 109 

 110 

The other case is KS mimicking vasculitis at the time of presentation. KS is a malignant tumor 111 

that affects immunocompromised patients such as those infected with HIV, those who receive 112 

immunosuppressants drugs, and those with congenital causes (8). KS skin lesions manifest as 113 

red, purple, and brownish patches and dots, which can be misidentified as malignant or vascular 114 

lesions in some phases due to the rise in superficial vascularity (9). 115 

 116 

p-ANCA, an important marker for ANCA-associated vasculitis, has been found to be 91% 117 

specific (10). The clinical pictures of a brown, purpuric rash on the lower limbs, as in our case, in 118 

addition to positive p-ANCA are highly suggestive of vasculitis. A previously reported case of 119 

KS and positive p-ANCA was due to existing vasculitis (11). In fact, the patient developed KS 120 

after receiving an immunosuppression agent to treat vasculitis. But our case is unique in that p-121 

ANCA was positive at the time of presentation with no existent vasculitis. However, the 122 



 

 
 

possibility of incidental finding or our patient could develop vasculitis later is possible. To the 123 

best of our knowledge, there are no reported cases in the literature of KS associated with positive 124 

p-ANCA with no existing vasculitis. Further studies, which take these variables into account, 125 

will need to be undertaken. 126 

 127 

Conclusion 128 

Many conditions can mimic the clinical and laboratory features of vasculitis. Herein, we present 129 

two cases of CES and KS as mimics that might delay the correct diagnosis. However, a previous 130 

history of angiography catheterization and biopsy would cut doubt with certainty. This study 131 

aims to raise awareness of the possible differential diagnoses of vasculitis. Further studies are 132 

needed to investigate the relation between positive p-ANCA in patients and KS. 133 

 134 

Acknowledgement 135 

The authors appreciate the Research Code Team (RCT) at Umm Al-Qura University (UQU) for their 136 

valuable contribution and efforts in supervising this research project. 137 

 138 

Authors’ Contribution 139 

ASA is responsible for the majority of the work. AMAm drafted the manuscript and handled the 140 

designing and formatting. WAA and AMAh collected the clinical information and wrote the initial 141 

manuscript. AmAA provided the histopathology results. AMAm formulated the abstract. MHA, AM, and 142 

AbAA were the treating physicians. All authors approved the final version of the manuscript. 143 

 144 

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Figure 1: the dermatological findings at presentation in case 1 175 

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Figure 2: the dermatological findings at presentation in case 2.  178 

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Figure 3: A:   shows areas of hemorrhage and vague network of connecting channels, dilated 181 

channels lined by small hyperchromatic nuclei B: HHV-8 immunohistostaining shows positive 182 

granular nuclear staining 183