Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from systemic non-specific features such as fever, malaise, arthralgia, and myalgia to specific organ damage. We describe two cases of cholesterol embolization syndrome and Kaposi sarcoma mimicking primary systemic vasculitis, both of which were characterized by features such as livedo reticularis, blue toe syndrome, a brown, purpuric skin rash, and positive p-ANCA associated with Kaposi sarcoma. Establishing the right diagnosis was&nbsp;challenging, and thus we&nbsp;aim in this study to highlight the possible ways to distinguish them from&nbsp;primary systemic vasculitis.
Keywords: Dermatological lesions, Cholesterol embolization syndrome, Kaposi sarcoma, vasculitis mimic

Ahmed, Amal A

Ahmed, Walaa A

Alhazmi, Abdullah M

Almalki, Abdulrahman M

Alqurashi, Abdulmohsen S

Alshehri, Abdulrahman A

Aly, Mohammed H

Mohammed, Abdulghaffar

English

Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from systemic non-specific features such as fever, malaise, arthralgia and myalgia to specific organ damage. We describe two cases of cholesterol embolisation syndrome and Kaposi sarcoma mimicking primary systemic vasculitis, both of which were characterised by features such as livedo reticularis, blue toe syndrome, a brown purpuric skin rash and positive perinuclear anti-neutrophil cytoplasmic antibodies associated with Kaposi sarcoma. Establishing the right diagnosis was challenging and thus this report aimed to highlight the possible ways to distinguish them from primary systemic vasculitis.
Keywords: Livedoid Vasculopathy; Cholesterol Embolisms; Blue Toe Syndrome; Kaposi Sarcoma; Cutaneous Vasculitis; Case Report; Saudi Arabia

Sultan Qaboos University Scientific Journals

   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 References 145 1.  Chen KR, Carlson JA. Clinical approach to cutaneous vasculitis. Am J Clin Dermatol. 146 2008;9(2):71–92.  147 2.  Shavit E, Alavi A, Sibbald RG. Vasculitis—What Do We Have to Know? A Review of 148 Literature. Int J Low Extrem Wounds [Internet]. 2018;17(4):218–26. Available from: 149 https://doi.org/10.1177/1534734618804982 150 3.  Mishra A, Iyadurai R, George A, Rajdurai E, Surekha V. Etiological and 151 clinicopathological study of secondary small vessel vasculitis in elderly: A case series of 152 12 patients. J Fam Med Prim Care. 2017;6(1):106.  153 4.  Sack KE. The difficulties of differentiating vasculitis from its mimics. Cleve Clin J Med. 154    1998;65(10):550–2.  155 5.  Molloy ES, Langford CA. Vasculitis mimics. Curr Opin Rheumatol. 2008;20(1):29–34.  156 6.  Fegan CD. Lesson of the Week: Any Questions. Bmj. 1995;310(6979):580.  157 7.  Maningding E, Kermani TA. Mimics of vasculitis. Rheumatol (United Kingdom). 158 2021;60(1):34–47.  159 8.  Vangipuram R, Tyring SK. Epidemiology of Kaposi sarcoma: review and description of 160 the nonepidemic variant. Int J Dermatol. 2019;58(5):538–42.  161 9.  Marušić Z, Billings SD. Histopathology of Spindle Cell Vascular Tumors. Surg Pathol 162 Clin. 2017 Jun;10(2):345–66.  163 10.  Guchelaar NAD, Waling MM, Adhin AA, van Daele PLA, Schreurs MWJ, Rombach SM. 164 The value of anti-neutrophil cytoplasmic antibodies (ANCA) testing for the diagnosis  of 165 ANCA-associated vasculitis, a systematic review and meta-analysis. Autoimmun Rev. 166 2021 Jan;20(1):102716.  167 11.  Fatma L Ben, Rais L, Mebazza A, Azzouz H, Beji S, Krid M, et al. Saudi Journal of 168 Kidney Diseases and Transplantation. Saudi J Kidney Dis Transplant [Internet]. 2013 169 [cited 2021 Nov 9];24(6):1199. Available from: 170 https://www.sjkdt.org/article.asp?issn=1319-171 2442;year=2013;volume=24;issue=6;spage=1199;epage=1202;aulast=Fatma 172  173  174 Figure 1: the dermatological findings at presentation in case 1 175  176     177 Figure 2: the dermatological findings at presentation in case 2.  178  179  180 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 

Dermatological Lesions of Cholesterol Embolisation Syndrome and Kaposi Sarcoma Mimic Primary Systemic Vasculitis: Case report study

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Dermatological Lesions of Cholesterol Embolization Syndrome and Kaposi Sarcoma Mimic Primary Systemic Vasculitis: Case Report Study

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