Job Syndrome Treatment & Management

Updated: Apr 05, 2016
  • Author: Jennifer Heimall, MD; Chief Editor: Michael A Kaliner, MD  more...
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Treatment

Medical Care

Care of patients with hyperimmunoglobulin E syndrome (HIES) is primarily directed at treating and preventing recurrent skin and sinopulmonary infections. Prophylactic antimicrobials, such as trimethoprim/sulfamethoxazole, are highly effective. With the increasing prevalence of antibiotic-resistant S aureus and other pathogens, it is important that microbial culture sensitivities be actively obtained to direct antimicrobial therapies. As patients often have minimal signs of systemic toxicity, the threshold for considering active infection requiring aggressive treatment should be low.

The control of dermatitis is essential to decrease the frequency of skin infections and improve the quality of life, particularly for pediatric patients with more severe dermatitis. Exposure to low concentrations of bleach, such as achieved from frequent swimming in a chlorinated pool or taking bleach baths (1/2 cup of bleach to a tub of water for 15 minutes, 3 times a week) are highly effective for controlling staphylococcal skin infections.

Mucocutaneous candidiasis and onychomycosis are treated as needed with antifungals therapy, most commonly fluconazole. Broadening antifungal coverage to include Aspergillus species (itraconazole, voriconazole, posaconazole) should be considered for individuals with pneumatoceles. Annual flu vaccine is recommended.

Intravenous immunoglobulin (IVIG) therapy has been used anecdotally in selected patients with improvement in infections.

Currently, no clear recommendations exist for routine medical therapy of the vascular, musculoskeletal, and connective tissue abnormalities associated with the disorder. Immune reconstitution would theoretically be of benefit for HIES, but the benefit to repairing the immune dysfunction while leaving the somatic abnormalities intact in these patients is unknown. Bone marrow or stem cell transplant has been used in too few cases to be sure of its safety or value in HIES.

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Surgical Care

The most common surgical procedure needed is incision and drainage of skin boils, which can often be performed in the office with local anesthesia. Extraction of primary teeth because of delayed deciduation is also common. Some patients with severe scoliosis require bracing and surgical treatment depending upon the degree of curvature. Rarely, patients require lobectomy or pneumonectomy because of pneumatoceles with recurrent infection. However, surgical resection of pneumatoceles should be undertaken only with expert advice, as patients may experience difficulty with residual lung expansion to fill the intrathoracic space with attendant medical complications.

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Consultations

Consultation with dermatology may be useful in management of HIES-associated eczematous dermatitis.

Consultation with infectious diseases may be useful in management of extensive pulmonary or cutaneous infection.

Consultation with pulmonology for bronchoscopy with culture may help diagnose the etiology of pneumonia. Bronchoscopy may also help clear thick secretions, as patients tend to have impaired mucociliary clearance.

Consultation with dentistry is often needed for assessment and treatment of retained primary teeth.

Consultation with orthopedics is commonly needed for evaluation and management of scoliosis and fractures.

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Diet

No dietary restrictions are necessary for HIES patients.

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Activity

No activity restrictions are necessary for HIES patients. However, many patients report chronic joint pain with aging, which may lead to activity restriction.

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