SHM Converge Scientific Abstract ePoster Gallery-Reactivated Viral Infections With Ophthalmologic/Cutaneous Manifestations in Undiagnosed Job's Syndrome

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This case report describes a 36-year-old man with chronic herpes simplex virus-1 and varicella zoster virus infections, recurrent eye disease, eczema, and prior corneal transplant who presented with worsening periocular and facial vesicular lesions in the V1 distribution, plus lesions on the neck and arms. Because of concern for herpes reactivation with possible ocular involvement, he was treated with IV acyclovir and ophthalmic erythromycin. Although viral cultures were negative, corneal cultures grew oxacillin-resistant Staphylococcus aureus.<br /><br />Further evaluation revealed markedly elevated IgE (21,686 IU/mL) and a history of recurrent childhood infections requiring hospitalization and IV antibiotics, raising suspicion for autosomal dominant hyper-IgE syndrome (AD-HIES, Job’s syndrome). AD-HIES is an immunodeficiency characterized by very high IgE, recurrent sinopulmonary and skin infections, eczematous dermatitis, and other classic findings such as newborn rash, bone fractures, characteristic facial features, and a high-arched palate. STAT3 loss-of-function mutations are the most common cause.<br /><br />The report emphasizes that recurrent viral infections, especially herpes zoster ophthalmicus and HSV, are unusual when combined with staphylococcal skin infections and should prompt a thorough immunologic workup, particularly in undiagnosed older patients. The patient was discharged on TMP-SMX, acyclovir, gabapentin, and ophthalmic erythromycin, with referral to immunology for confirmatory testing, including T-cell mitogen proliferation studies and STAT3 genetic analysis.<br /><br />Overall, the case highlights the importance of recognizing atypical ophthalmic and cutaneous infections as potential clues to underlying immunodeficiency, and of managing AD-HIES with careful skin care and prophylactic antimicrobial therapy.

Asset Subtitle

Andrea C. Lin

Meta Tag

Author List Andrea C. Lin, Jonathan Nunez, Sarah Al-Obaydi, Vraj Shah

Category Clinical Vignettes

Concept Immunodeficiency

Concept Autosomal Dominant Hyper-IgE Syndrome

Concept Elevated IgE

Concept Recurrent Infection

Concept Pneumonia

Distinguished Non-Finalist

Presenter Organization Penn State College of Medicine

Presenting Author Andrea C. Lin

Track Adult

Keywords

hyper-IgE syndrome

STAT3 mutation

herpes simplex virus

varicella zoster virus

herpes zoster ophthalmicus

staphylococcal infection

eczema

immunodeficiency

corneal transplant

elevated IgE

Immunodeficiency

Autosomal Dominant Hyper-IgE Syndrome

Elevated IgE

Recurrent Infection

Pneumonia