A 13-year-old girl who had received the first and second doses of the Sinopharm COVID-19 vaccine on October 9, 2021, and November 8, 2021, respectively, presented with gangrene-like symptoms starting from the forefinger of the left hand with painful and tingling sensations (Figs. 1 & 2). Due to the exacerbation of the symptoms, the patient was admitted to Namazi Hospital, Shiraz, Iran, on March 18, 2022. She received nifedipine and alprostadil due to Raynaud’s phenomenon and got better, and left the hospital against medical advice on April 3, 2022.
The necrosis symptoms exacerbated and reached both feet and hands, so the patient was admitted again on Apr 11, 2022 (Fig. 3). Physical examination revealed high blood pressure (right femur 175/128 mmHg, right leg 166/115 mmHg, left femur 186/132 mmHg, left bp 180/129 mmHg, right arm 138/131 mmHg, and left arm 141/104 mmHg). The blood pressure remained high despite administering enalapril and diltiazem but was finally controlled with the addition of clonidine. We treated the patient with alprostadil (5 days; later changed to bosentan), aspirin, fresh frozen plasma, enoxaparin, diltiazem, valsartan, clonidine, and gabapentin. Abnormal lab tests included ANA (Hep2): 3 U, Anti-ds-DNA: 7.6 U/mL, SARS-CoV-2 IgG: 5.3AU/mL, anti-SARS-CoV-2 IgG: 7.93 AU/mL (This lab data check for 2 times), Anti-Cardiolipin Ab IgG: 12 U/mL, protein C: 163 IU Dl-1, anti-RNP/Sm: 17 U, CENP: 40 AU/mL, PTT:27.6 Second, PT:142 Second, ALT:100U/L then 30 U/L, AST: 92 U/L then 17 U/L (Table 1).
Table 1
Test
|
Result (first)
|
Result (second)
|
Lupus anti coagulant
|
33.5 MPL Units
|
|
Protein C
|
10 IU Dl-1(normal)
|
|
Protein S
|
97%(normal)
|
|
Factor V Leiden
|
2.5%(normal)
|
|
Anti thrombin 3
|
110 Mg/dlNormal
|
|
CH 50
|
130 U/mL(normal)
|
|
SARS-COV-2 IgG
|
5.3 AU/mL (positive)
|
|
SARS-COV-2 IgM
|
0.4 IU/mL(normal)
|
|
c-ANCA
|
1.9 U/mL (negative)
|
|
p-ANCA
|
1 U/mL (negative)
|
|
Anti-cardiolipin Ab(IgG)
Anti-cardiolipin Ab(IgM)
|
12 U/mL(normal)
1.3 U/mL(normal)
|
|
Anti-phospholipid Ab(IgM)
Anti-phospholipid Ab(IgG)
|
0.4 U/mL(normal)
3.8 U/mL(normal)
|
|
Anti Beta 2 Glycoprptein IgM
Anti Beta 2 Glycoprptein IgG
|
1.1U/Ml(Normal)
0.3 U/mL(Normal)
|
|
ANA (Hep 2)
|
3 U
|
|
Anti-ds-DNA
|
7.6 U/mL (normal)
|
|
White blood cells
|
12000 mm3
|
|
Hemoglobin
|
10.5 g/dL
|
|
Platelets
|
375 10*3/uL
|
|
Blood urea nitrogen
|
20 mg/dl
|
|
Creatinine
|
0.6 mg/dl
|
|
Sodium
|
143 mEq/L
|
|
Potassium
|
4 mEq/L
|
|
C-reactive protein
|
24 mg/dl
|
|
Calcium
|
9 mg/dl
|
|
Magnesium
|
2 mg/dl
|
|
Erythrocyte sedimentation rate
|
33 mm/hr
|
|
Aspartate aminotransferase
|
23 U/L
|
|
Prothrombin time
|
15 second
|
|
Activated partial thromboplastin time
|
33 second
|
|
International normalized ratio
|
1.2
|
|
D-dimer
|
2185 ng/ml
|
|
Creatine phosphokinase
Homocystein
C3
C4
IgE
IgM
IgG
IgA
RF
Anti dsDNA
Anti-SS-A
Anti-SS-B
Anti-Jo-1
Anti RNP/SM
Anti Centromer Ab(CENP)
Anti-Scl-70
|
500 IU/L
9 U mol/L
1.07 g/l
0.17 g/l
132 Iu/ml
0.98 g/l
15.34 g/l
2 g/l
8 Iu/ml
7.6 U/ml
2 unite/ml
1 unite/ml
1 U
17 U
40 Au/ml
1%
|
|
Paraclinical workups such as color Doppler sonography of both upper extremities arteries and veins were normal. No signs of deep venous thrombosis (DVT) or thrombosis were detected in the color Doppler sonography of both lower extremities arteries and veins. the normal function of both kidneys was approved by DSMA renal scintigraphy. Magnetic resonance imaging (MRI) of the brain without and with contrast suggested a metabolic disorder due to excessive basal ganglia calcification. Also, magnetic resonance angiography of the brain was normal. Spiral computed tomography (CT) scans of the chest, mediastinum, abdomen, and pelvis were normal. Spiral CT angiography (CTA) of the lower extremities, thoracic aorta, and abdominal aorta were normal; however, vasculitis and narrowing of the radial and ulnar arteries were evident on the left upper extremity. Pathological examination of the specimen from a punch biopsy of the skin suggested acute neutrophilic leukocytoclasis vasculitis involving small blood vessels. Bone marrow aspiration biopsy noted moderate hypocellular marrow.
For controlling the high blood pressure we ordered diltiazem, bosentan, hydrochlorothiazide, tadalafil, and L-arginine. After consulting with a fellow in pain medicine, clonidine was also added. Note that pentoxifylline and cilostazol were not available at that time in our country. The patient did not respond well, so further management included a single dose of cyclophosphamide, 2 doses of rituximab, 5 doses of plasmapheresis (QOD), vasodilators, and sympathetic blockade. Accordingly, the disease progress stopped, and a positive response to the treatment method was achieved.