Purpura Fulminans After Varicella Infection and Pulmonary Embolism After COVID-19 Infection in Familial Mediterranean Fever: Coincidence or Not?

anemia (hemoglobin 9.1 g/dL), normal platelet count (213 × 10 3 /UL), decreased lymphocyte count (1100/mm 3 ), elevated neutrophil count (9900/mm 3 ), and elevated acute-phase reactants (C-reactive protein 308 mg/dL and erythrocyte sedi-mentation rate 94 mm/h). Urinalysis, renal, and liver function tests were all normal. Penile arterial Doppler ultrasonography revealed a decrease in blood flow in the cavernous bod-ies. In coagulation parameters, prothrombin time (PT) was prolonged (15.7 seconds) and D-dimer level was elevated (18.8 µg/mL), but activated partial thromboplastin time (25.6 seconds) and fibrinogen (301 mg/dL) levels were normal. Protein C (PC) (64%), protein S (PS) (34%), and anti-thrombin-3 (AT-3) activity (42%) levels were all decreased and then normalized after 6 weeks from PF. Inherited risk factors (factor V Leiden and prothrombin G20210A) for thrombosis were screened and excluded. Neither the patient nor her family had a history of thrombophilia. Anti-nuclear antibody, antiphospholipid antibodies, anti-neutrophilic cytoplasmic antibody, rheumatoid factor, and human leukocyte antigen-B51 (HLA-B51) were negative, while complement levels were normal. Fresh-frozen plasma trans-fusion with empirical antibiotic therapy was started. During the follow-up, swelling on the legs developed, and deep-vein thrombosis was detected in the bilateral vena saphenous

[1]  Neethu M. Menon,et al.  Thromboembolism in children with multisystem inflammatory syndrome: a literature review , 2022, Pediatric Research.

[2]  C. Linari,et al.  SARS‐COV‐2–associated coagulopathy and thromboembolism prophylaxis in children: A single‐center observational study , 2020, Journal of Thrombosis and Haemostasis.

[3]  Y. Hu,et al.  Clinical features of patients infected with 2019 novel coronavirus in Wuhan, China , 2020, The Lancet.

[4]  Simin Zhang,et al.  Purpura fulminans from reduced protein S following cytomegalovirus and varicella infection , 2019, American journal of hematology.

[5]  E. Ben-Chetrit,et al.  Familial Mediterranean Fever and Hypercoagulability , 2011, Mediterranean journal of hematology and infectious diseases.

[6]  I. Uthman,et al.  Childhood stroke in a child with familial Mediterranean fever carrying several prothrombotic risk factors , 2009, Lupus.

[7]  A. Ozkan,et al.  Is Familial Mediterranean Fever a thrombotic disease or not? , 2008, European Journal of Pediatrics.

[8]  K. Kavaklı,et al.  Hypercoagulability: interaction between inflammation and coagulation in familial Mediterranean fever , 2007, Clinical Rheumatology.

[9]  C. Esmon Inflammation and thrombosis , 2003, Journal of thrombosis and haemostasis : JTH.

[10]  A. Zaglio [Update on familial Mediterranean fever]. , 1995, Revista de medicina de la Universidad de Navarra.

[11]  R. Marlar,et al.  Proposed Classification and Pathologic Mechanisms of Purpura Fulminans and Skin Necrosis , 1990, Seminars in thrombosis and hemostasis.

[12]  D. Jatužis,et al.  The Role of Colchicine in the Prevention of Cerebrovascular Ischemia. , 2018, Current pharmaceutical design.

[13]  O. Kasapcopur,et al.  Familial Mediterranean fever in childhood: a single-center experience , 2017, Rheumatology International.