Herpes Zoster Infection Prompting Diagnosis of Job's Syndrome in a Teenage Patient

Rationale: Job’s syndrome or autosomal dominant hyperimmunoglobulin E syndrome (AD-HIES) occurs due to defects in signal transducer and activator of transcription-3 (STAT3) and is characterized by staphylococcal abscesses, sinopulmonary infections, pneumatoceles, elevated IgE, candidiasis, bone fractures, delayed loss of primary teeth, and eczema. Primary viral infections are uncommon in AD-HIES, but varicella zoster virus (VZV) reactivation occurs and is associated with central memory T cell defects. We present the case of a teenager with severe ocular and cutaneous herpes zoster (HZ), the severity of which prompted an exhaustive review of medical records and led to the correct diagnosis.  

Methods: A retrospective chart review was performed.

Results: An 18 year old female developed severe ocular and cutaneous HZ involving V1 and V2 of the trigeminal nerve with secondary pre-septal cellulitis and visual field defects. She had a remote history of elevated IgE. Review of records revealed frequent pneumonia with pneumothorax in one case and development of a pneumatocele, recurrent otitis media, cutaneous abscesses, manual extraction of all primary teeth, and elevated IgE (17,900IU/ml). Her mother died from disseminated coccidiodmycosis and pulmonary aspergillosis. Her NIH Job’s clinical feature score was 42, suggestive of AD-HIES and was molecularly confirmed with a heterozygous missense substitution in exon 21 (c.1915C>T) of STAT3. Treatment with empiric antibiotics and prolonged intravenous acyclovir led to gradual improvement.

Conclusions: Primary viral infections occur rarely in patients with AD-HIES, however, VZV reactivation is not uncommon. HZ infection, especially in a young patient, should prompt a detailed infectious history and raise concern for primary immunodeficiency.