In addition, severe recur- rent fungal and viral infections including mucocutaneous candidiasis, molluscum contagiosum, herpes zoster and herpes simplex were noted in some patients
📌 3 Key Takeaways
🎯
Objective & Hypothesis:Hyperimmunoglobulin E syndrome HIES has been descri- bed as a primary immunodeficiency and multisystem disor- der characterized by elevated serum IgE levels, recurrent skin abscesses, pulmonary infections, eczematous dermatitis, skeletal and dental abnormalities.
📊
Key Findings & Data:Hyperimmunoglobulin E syndrome HIES has been descri- bed as a primary immunodeficiency and multisystem disor- der characterized by elevated serum IgE levels, recurrent skin abscesses, pulmonary infections, eczematous dermatitis, skeletal and dental abnormalities.
💡
Conclusion & Impact:In addition, severe recur- rent fungal and viral infections including mucocutaneous candidiasis, molluscum contagiosum, herpes zoster and herpes simplex were noted in some patients
Hyperimmunoglobulin E syndrome HIES has been descri- bed as a primary immunodeficiency and multisystem disor- der characterized by elevated serum IgE levels, recurrent skin abscesses, pulmonary infections, eczematous dermatitis, skeletal and dental abnormalities. In addition, severe recur- rent fungal and viral infections including mucocutaneous candidiasis, molluscum contagiosum, herpes zoster and herpes simplex were noted in some patients
Özet
Hiperimmunglobulin E sendromu HİES , artmış serum IgE düzeyleri, yineleyen cilt abseleri akciğer enfeksiyonla- rı, egzamatöz dermatit, iskelet ve diş anormallikleri ile karakterize primer immun yetersizlik ve multisistem hasta- lığı olarak tanımlanır. Ayrıca hastaların bazılarında muko- kütanöz kandida enfeksiyonu, molloskum kontagiozum, herpes zoster, herpes simpleks gibi ağır, yineleyici mantar ve virüs enfeksiyonları gösterilmiştir
DEMİR, A. D., ARI, E., UZUNER, S., GEDİK, A. H., GÖKNAR, N., ERENBERK, U., ÇAKIR, E. (2015). Disseminated Molluscum Contagiosum in Two Siblings with Hyperimmunglobulin E Syndrome. Çocuk Dergisi, Cilt: 15 - Sayı: 2, 82–85. https://ajindex.com/articles/246868
Grimbacher b, Schäffer aa, Holland SM, Gallin Jı, Malech Hl, atkinson TP, et al. Genetic linkage of hyper-IgE syndrome to chromosome 4. Am J Hum Genet 1999;65:735-44. http://dx.doi.org/10.1086/302547
Tabel Y, Mungan i, akta şM, karadağ n, kırımlıoğlu H. Hiperimmünglobulin E sendromlu sekiz yaşında erkek çocukta nefrotik sendrom: Vaka sunumu. Çocuk Derg 2008;8:117-9. kılıç Sş. İmmün yetmezlikli hastalarda görülen enfek- siyonlar. Güncel Pediatr 2005;2:40-6.
Çınar S, Gelmez MY, nepesov S, Camcıoğlu Y, Deniz G. Hiperimmünoglobulin E sendromlu hastalar- da DOCK8 düzeyleri: Akan hücre ölçer ile saptanması. Turk J Immunol 2015;3(2):71-5. http://dx.doi.org/10.5606/tji.2015.424 zhang Q, Davis JC, lamborn ıT, Freeman aF, Jing H, Favreau aJ, et al. Combined immunodeficiency associated with DOCK8 mutations. N Engl J Med 2009;361:2046-55. http://dx.doi.org/10.1056/NEJMoa0905506 kharkar V, kardekar S, Gutte r, Mahajan S, Thakkar V, khopkar U. Disseminated molluscum contagiosum infection in a hyper IgE syndrome. Indian J Dermatol Venereol Leprol 2012;78:371-4. http://dx.doi.org/10.4103/0378-6323.95464 engelhardt kr, McGhee S, Winkler S, Sassi a, Woellner C, lopez-Herrera G, et al. Large deletions and point mutations involving the dedicator of cytokinesis 8 (DOCK8) in the autosomal-recessive form of hyper-IgE syndrome. J Allergy Clin Immunol 2009;124:1289-302. http://dx.doi.org/10.1016/j.jaci.2009.10.038 renner eD, Puck JM, Holland SM, Schmitt M, Weiss M, Frosch M, et al. Autosomal recessive hype- rimmunoglobulin E syndrome: a distinct disease entity. J Pediatr 2004;144:93-9. http://dx.doi.org/10.1016/S0022-3476(03)00449-9 karadağ aS, akdeniz n, Çalka ö, bilgili SG, altun F. Hiperimmunglobulin E sendromlu bir vaka. Van Tıp Derg 2012;19:126-9.