P079 Multi-systemic inflammatory syndrome in children: skin tropism

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Rahma Guedri ◽  
Mariem Glai ◽  
Zohra Fitouri ◽  
Saayda Ben Becher

Abstract Background Multisystem inflammatory syndrome in children (MIS-C) is a severe immune-mediated syndrome that occurs after COVID-19 infection. It mainly affects children and presents several clinical aspects. The cutaneous and mucous signs are very much part of the diagnostic criteria. The aimisto describe the clinical and evolutionary aspects of the muco-cutaneous signs during MIS-C Patients and methods It was a descriptive retrospective study conducted over a period of 9 months (March 1, 2020 - December 31, 2020) including children admitted to COVID isolation unit with suspicion of MIS-C syndrome. Results We included 17 patients. The average age was 7 years (1–11 years). The sex ratio was 2.2 (11 boys and 6 girls). There was o history of dermatological disease has been reported. Fever was present in all patients. Mucosal signs were present in 13 patients. Conjunctival hyperemia without purulent secretions was noted in 12 patients. Conjunctivitis was bilateral in 11 cases. A rash was found in 10 cases. It was macular (6 cases), maculopapular (2 cases) and vesicular cluster in one 1 case. It was itchy in 2 cases. It was located in the limbs (7 cases), thorax and/or abdomen (5 cases), pelvis (4 cases), palms and/or soles (3 cases) and the face in one case. Cheilitis was found in 6 cases and stomatitis or glossitis in 5 cases. oEdema of the extremities was present in three patients and oedema of the face was noted in one patient. All children received intravenous immunoglobulin therapy combined with high-dose corticosteroids and acetylsalicylic acid at anti-aggregating doses. The course was marked by the disappearance of the muco-cutaneous signs without recurrence in all cases. Conclusion Mucocutaneous involvement is a characteristic manifestation of multisystem inflammatory syndrome. It is one of the various diagnostic criteria for this syndrome.

1997 ◽  
Vol 245 (1) ◽  
pp. 26-31 ◽  
Author(s):  
J. L. M. Jongen ◽  
P. A. van Doorn ◽  
Frans G. A. van der Meché

2006 ◽  
Vol 148 (3) ◽  
pp. 399-400 ◽  
Author(s):  
Yasunori Wada ◽  
Atsushi Kamei ◽  
Yukiharu Fujii ◽  
Ken Ishikawa ◽  
Shoichi Chida

Blood ◽  
1998 ◽  
Vol 91 (10) ◽  
pp. 3623-3629 ◽  
Author(s):  
Hiroshi Mohri ◽  
Shigeki Motomura ◽  
Heiwa Kanamori ◽  
Michio Matsuzaki ◽  
Shin-ichiro Watanabe ◽  
...  

Abstract Of 260 patients enrolled, 25 patients (9.6%) were associated with acquired von Willebrand syndrome (AvWS). We studied 25 patients with AvWS, retrospectively. AvWS was diagnosed by reduced levels of von Willebrand factor (vWF) (decrease of von Willebrand factor antigen [vWF:Ag] and von Willebrand ristocetin cofactor [vWF:RCoF]), a decrease of ristocetin-induced platelet agglutination (RIPA), sometimes decreased high-molecular-weight multimers, and prolonged bleeding time with neither prior nor family histories of bleeding problems and the evidence of normal vWF:RCoF in their families. The inhibitor of vWF was determined by mixing patient plasma with pooled normal plasma. Eight patients in this study had the inhibitors to vWF that were of the IgG class; the subclasses were IgG1 (7 cases) and IgG2 (1 case). Multimeric analysis of vWF showed selective loss of large multimers in most patients with AvWS similar to that of congenital type-2 von Willebrand disease (vWD). All inhibitors blocked ristocetin-mediated vWF binding to platelets. Five out of 6 IgGs evaluated here recognized the 39/34-kD fragment (residues 480/481-718) and Fragment III (residues 1-1365) that implied binding domain of glycoprotein Ib (GPIb), whereas 1 recognized Fragment I (residues 911-1365). A close relationship was found between the presence of the inhibitor and bleeding tendency. Of the 7 patients with inhibitors, 6 patients (86%) had a bleeding tendency, as well as 1 of the 15 patients without inhibitors (6%). The efficacy of treatment of underlying diseases and/or therapy with deamino D-arginine vasopressin (DDAVP) for the treatment of AvWS also depends on the presence of an inhibitor. Four of 8 patients with inhibitors (50%) had poor response to treatment of the underlying disease and/or therapy with DDAVP, as well as 1 of the 16 patients without inhibitors (6%). These results indicate that patients with AvWS developing inhibitors to vWF are likely to have bleeding problems and might be resistant to treatment of underlying diseases and/or therapy with DDAVP for bleeding to AvWS. We also showed evidence that intravenous immunoglobulin therapy (0.3 g/kg, 3 days) was effective to correct a hemostatic defect and manage severe bleeding in a patient with AvWS developing inhibitors. We might consider an additional treatment including expensive high-dose immunoglobulin therapy when uncontrollable bleeding is continued after the treatment of the underlying diseases and/or therapy with DDAVP.


2006 ◽  
Vol 119 (14) ◽  
pp. 1230-1232 ◽  
Author(s):  
Xi-bao ZHANG ◽  
Yu-qing HE ◽  
Hua ZHOU ◽  
Quan LUO ◽  
Chang-xing LI

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