Dermatologic Manifestations of Job Syndrome Treatment & Management

Updated: Mar 30, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

No definitive therapy is available for the treatment of hyper-IgE syndrome (HIE syndrome or Job syndrome). The mainstay of treatment is the control of bacterial infections. Early incision and drainage followed by the intravenous administration of antibiotics are used for cutaneous infections. Coverage is usually aimed at Staphylococcus and Haemophilus species. The second-generation cephalosporin cefaclor has also been recommended. [5]

Acupuncture treatment has been reported to be of value for symptom management of patients with hyper-IgE syndrome, although further studies are required for confirmation. [47]

Job syndrome therapy is usually longer than typical treatment because the disease in these patients responds more slowly than that of patients without Job syndrome. Intravenous antibiotic treatment for 2 weeks is typical. Chronic onychomycosis responds well to oral ketoconazole and fluconazole. Eczematous dermatitis has a varied response to high-dose topical steroids.

Chemoprophylaxis in patients with Job syndrome has varied results. Levamisole, an immunopotentiating drug, has been investigated as a therapeutic agent; in one study, it was unhelpful. Long-term trimethoprim-sulfamethoxazole treatment was used in one patient with recurrent pruritic dermatitis, with resolution of symptoms. [48] Other patients treated with prophylactic antibiotics had both minor and major infections during therapy, often after several months of being infection free.

Cases in patients with severe hyper-IgE syndrome whose disease was unresponsive to other therapeutic modalities are reported; these cases had a marked clinical response to cyclosporin A. Treatment included low-dose cyclosporin for 6 months or longer. Both cutaneous and pulmonary infections responded to this therapy, and no adverse effects were reported. In one study, oral disodium cromoglycate (2 g/d) prevented the complications of Job syndrome over a 2-year period. [49] Two case studies in patients with Job syndrome have shown a dramatic response in preventing infectious and eczematoid complications; patients were treated for as long as 18 months.

In one open-labeled study, high-dose intravenous immunoglobulin had no clear clinical benefit in 9 patients with Job syndrome. Another study showed an improvement in severe eczema along with a decrease in serum IgE levels in 2 patients after they were treated with high-dose intravenous gamma globulin. [50]

The autosomal recessive variant may benefit from bone marrow transplantation and hematopoietic stem cell graft. [51, 52]

Acupuncture treatments may decrease the severity of symptoms. [53]

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Surgical Care

Surgical excision and drainage of cutaneous infections are often performed in patients with Job syndrome (HIE syndrome, or hyper-IgE syndrome). Drainage is usually followed by intravenous antibiotic therapy.

Chronic hidradenitis suppurativa occurs in some patients with Job syndrome. Often, these lesions do not respond to antibiotics, and local excision may be required.

Special care is required for surgical procedures in these patients. [54]

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Consultations

An allergist and immunologist may help in establishing the diagnosis of Job syndrome (HIE syndrome, or hyper-IgE syndrome). An infectious disease specialist may help in cases with infectious complications. An orthopedist should be involved in the care of those with scoliosis and fractures.

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Prevention

Initiate treatment at the first signs of infection to prevent long-term complications from Job syndrome (HIE syndrome, or hyper-IgE syndrome). Regularly screen Job syndrome patients for scoliosis so that early noninvasive treatment can be used.

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Long-Term Monitoring

Extra vigilance may be required in routine screening of Job syndrome (HIE syndrome, or hyper-IgE syndrome) patients for scoliosis. If a school-based program exists, healthcare providers should be made aware of the Job syndrome patient's greater-than-typical risk of scoliosis. Early detection with proper care can prevent progression of Job syndrome.

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