Curr Opin Hematol
Curr Opin Hematol. dermatitis persisted despite an aggressive treatment regimen. The serum IgE level increased exponentially over a period of 7 years, with a peak value of 57,400 IU/ml. Molecular genetic testing revealed a dominant unfavorable mutation within the SH2 domain name of the Transmission Transducer and Activator of Transcription (STAT3) gene. The patient was subsequently diagnosed with Jobs syndrome. Management included proper skin care, prophylactic antibiotics, immunomodulating agents, and psychotherapy. Conclusions: Jobs syndrome can often go unrecognized and masquerade as atopic dermatitis. Therefore, genetic testing for this condition should be obtained in all patients with treatment-refractory AD. Additionally, psychotherapy can be a successful management strategy for the grating psychological impact that can be imposed on children with excessive pruritus. and [10]. The resolution of pneumonia after administration of IVIG in Jobs syndrome has been described [11]. However, 1 study concluded that IVIG was of no clear benefit, and did not significantly decrease serum IgE Rabbit Polyclonal to AKAP14 levels or IgE synthesis [12]. IFN-gamma has demonstrated effectiveness in decreasing serum IgE levels [13]. However, serum IgE levels returned to pre-treatment levels within 1C3 months after completion of treatment [13]. In addition to these treatments, psychotherapy was an integral part of the patients treatment regimen. em Dr. Maria Elena Abdulmassih /em : I initially met this patient after he experienced an episode of intense Olaquindox agitation that caused him to scratch himself until he bled. During the course of family therapy sessions, I observed that this patient would use his constant state of pain and scratching to manipulate the parents into obeying him. The patient would use scratching and pain as a tool for social isolation. He did not want anyone to see him in his current state and also wished that he was dead. This led the parents to isolate the boy from normal social life. During family therapy, I was able to demonstrate to the parents that this defense mechanism of isolation was detrimental to the patients mental health. I was able to explain to the patient that blaming others for his condition was not helpful, and that he had to cope and hopefully overcome it in life. Reinforcing the ego of the patient was an integral part Olaquindox of the treatment in an effort to control his anger. The same mechanism of manipulating his parents was seen while bathing. Due to complaints Olaquindox of agonizing pain, he refused to bathe. This contributed enormously to the vicious cycle of his recurrent infections and secondary pruritus. After multiple interventions during family therapy, this patient began to demonstrate an improved attitude towards his condition. We were then able to start an aggressive plan consisting of daily showers, wound care, and hyperbaric oxygen therapy. Conclusions We conclude that further research is necessary regarding treatment regimens for Hyper-IgE syndrome (Jobs syndrome). This condition can be identical in presentation to AD; therefore, genetic testing should be obtained in all children with treatment refractory AD. Psychotherapy can be a successful management strategy for the severe psychological impact that can be imposed on children with excessive pruritus, in addition to pharmacological therapy. Footnotes Statement We have no sources of financial support and no conflicts of interest to disclose. References: 1. Job syndrome Genetics Home Reference [serial online] 2008. Feb, [cited 2015 Sep 10]. Available Olaquindox from: http://ghr.nlm.nih.gov/condition/job-syndrome. 2. Davis SD, Schaller J, Wedgewood RJ. Jobs Syndrome. Recurrent, cold, staphylococcal abscesses. Lancet. 1966;1(7445):1013C15. [PubMed] [Google Scholar] 3. Buckley RH, Wray BB, Belmaker EZ. Extreme hyperimmunoglobulinemia E and undue susceptibility to infection. Pediatrics. 1972;49(1):59C70. [PubMed] [Google Scholar] 4. Grimbacher B, Holland SM, Gallin JI, et al. Hyper-IgE syndrome with recurrent infections C an autosomal dominant multisystem disorder. N Engl J Med. 1999;340(9):692C702. [PubMed] [Google Scholar] 5. Farmand S, Sundin M. Hyper-IgE syndromes: recent advances in pathogenesis, diagnostics and clinical care. Curr Opin Hematol. 2015;22(1):12C22. [PubMed] [Google Scholar] 6. Bieber T. Atopic dermatitis. N Engl J Med. 2008;358(14):1483C94. [PubMed] [Google Scholar] 7. Massaad MJ, Ramesh N, Geha RS. Wiskott-Aldrich syndrome: A comprehensive review. Ann NY Acad Sci. 2013;1285:26C43. [PubMed] [Google Scholar] 8. Rivers L, Gaspar HB. Severe combined immunodeficiency: recent developments and guidance on clinical management. Arch Dis Child. 2015;100(7):667C72. [PubMed] [Google Scholar] 9. Cruz-Portelles A, Estopinan-Zuniga D. A new case of Jobs syndrome at the clinic: a diagnostic challenge. Rev Port Pneumol. 2014;20(2):107C10. [PubMed] [Google Scholar] 10. Kimata H. High-dose intravenous gamma-globulin treatment for hyperimmunoglobulinemia E syndrome. J Allergy Clin Immunol. 1995;95(3):771C74. [PubMed] [Google Scholar] 11. Bilora F, Petrobelli F, Boccioletti V, Pomerri F. Moderate-dose intravenous immunoglobulin treatment of Jobs syndrome. Case report. Minerva Med. 2000;91(5C6):113C16. [PubMed] [Google Scholar] 12. Wakim M, Alazard M, Yajima A, et al. High dose intravenous immunoglobulin.