Hyperimmunoglobulinemia E (Job) Syndrome Follow-up

Updated: Oct 21, 2015
  • Author: Harumi Jyonouchi, MD; Chief Editor: Russell W Steele, MD  more...
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Follow-up

Further Outpatient Care

Dental care must be provided when primary teeth fail to be shed and interfere with eruption of permanent teeth in patients with AD HIES.

There is on case report of the use of IFN-α for treatment of severe molluscum skin infection in one patient with HIES. [35]

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Further Inpatient Care

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  • Pneumonia in patients with HIES may be extremely complicated and require prolonged inpatient management. The abnormal inflammatory response with progression to pneumatoceles requires close observation and possible surgical intervention. Empyemas, bronchopleural fistulas, and hemoptysis caused by erosion into bronchial arteries are potential emergencies.

  • Other infections requiring inpatient care include osteomyelitis, which may be difficult to distinguish clinically from fractures, and deep-seated abscesses or myositis requiring incision, drainage, and packing.

  • When vigorous antibiotic therapy is required for infection, fungal prophylaxis is also required because most patients are at increased risk for mucocutaneous and invasive fungal infections, predominantly Candida and Aspergillus. Pay careful attention to signs of superinfection of lung abscesses with Aspergillus.

  • In patients with AR HIES, cutaneous viral infection can be very treatment-resistant and a prolonged treatment may be required.

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Inpatient & Outpatient Medications

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  • See Medical Care.

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Transfer

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  • Most clinical immunologists feel strongly that the great complexity of medical problems for any primary immunodeficiency disease requires treatment of those patients by an immunologist. Subtle signs of infection, complex clinical features, and high complication rates in patients with HIES suggest a vital role for a clinical immunologist for the care of patients with HIES.

  • A major reason for transfer is for thoracic surgery management of a lung abscess, bronchopulmonary fistula, or erosion of infection into a bronchial artery.

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Deterrence/Prevention

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  • Prophylactic oral antibiotic coverage for S aureus and an antifungal agent against Candida species are required for most patients.

  • Prenatal diagnosis may be possible in a child born to parents with know mutations with STAT3 or DOCK8.

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Complications

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  • Pulmonary complications of infection, such as bronchopulmonary fistula or bleeding, are surgical emergencies.

  • Craniosynostosis has been reported in several patients with AD HIES.

  • Occasional cases of malignancy have been reported mainly in AR-HIES patients, mainly originating from skin. Careful monitoring is indicates.

  • In 13 patients with AR HIES, 5 were reported to have CNS symptoms associated with vascular anomalies (stenosis, occlusion, and aneurysm formation), and 3 of 5 these patients died with subsequent complications (cerebral infarction and subarachnoid hemorrhages).

  • Recently, fatal aneurysmal dilatation of the thoracic aorta was reported in 2 adolescents with AR HIES. Coronary artery aneurysms were also reported in 2 patients with AD HIES who were in their fifth decade of life when aneurysms were diagnosed.

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Prognosis

Follow-up care for patients with HIES is not well documented, but most patients with AD HIES survive into mid adulthood. Chronic pulmonary disease compromises function and affects the mortality rate. Most deaths in the second to third decade of life result from lung abscesses superinfected with Aspergillus species or gram-negative bacteria. More aggressive medical and surgical care may decrease this mortality rate.

Failure of hematopoietic stem cell transplantation to correct HIES in one patient raises the question of whether therapy for HIES with any form of stem-cell reconstitution would be effective. This may also be associated with mutations causing HIES. Patients with DOCK8 deficiency may be expected to benefit from stem-cell reconstitution more so than other HIES patients.

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Patient Education

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  • Patients and their families must be alert to early subtle signs of infection and seek appropriate medical care. In the author's experience, primary care physicians and surgeons also often underestimate the extent of deep abscess formation and need for surgical drainage.

  • Daily care for eczema is tedious. Persuading patients and their families of the use of daily care is difficult when dermatitis does not respond uniformly to medical management.

  • The Immune Deficiency Foundation is an important resource for education and for support for patients and families with any primary immunodeficiency disease. The foundation's address is 40 W Chesapeake Ave, Suite 308, Towson, MD 21204; some states have local chapters. The telephone number for consultation calls is (800) 296-4433.

  • The Jeffrey Modell Foundation at 747 3rd Avenue, New York City, NY 10017 also provides support and patient education. The telephone number is (212) 819-0200.

  • For excellent patient education resources, visit eMedicineHealth's Skin Conditions and Beauty Center. Also, see eMedicineHealth's patient education article Eczema.

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