Facial impetigo and preseptal cellulitis associated with Job’s Syndrome, a rare hyperimmunoglobinemia E syndrome (HIES)

C Tong, OD; R Frick, OD, FAAO; D Hitchmoth, OD, FAAO

This case discusses the diagnosis and management of severe preseptal cellulitis caused by Job’s Syndrome. This disease compromises several body systems, especially the immune system, and causes recurrent infections.

I. Case History -67 year-old white male presents from the emergency department with marked erythema, edema and crusted lesions of both eyelids OD and face for 2 days -Medical history: Job’s Syndrome, hyperlipidemia and Neurofibromatosis Type 1 -Medications: Enoxaparin, Ibuprofen, and Simvastatin.

II. Pertinent findings -Best corrected vision: R 20/25 and L 20/30 -Marked honey-colored, encrusted, exudative lesions on upper and lower lids OD, left forehead, and extremities with OD swollen shut -Flat and raised, coffee colored lesions along extremities and trunk -No fever, lymphadenopathy or other systemic complaints -Pupils normal with no afferent defect -No proptosis -Extraocular muscles intact with no diplopia and no pain/paresthesia along cranial nerve V -Anterior and posterior segment unremarkable except iris lisch nodules OU -Computed tomography (CT) with contrast of the orbits found preseptal soft tissue changes [cellulitis] and normal post-septal space without other orbital abnormalities.

III. Differential Diagnoses - Preseptal Cellulitis, Herpes Zoster Ophthalmicus, orbital cellulitis, insect bite, hordeola, infectious dermatitis, allergic dermatitis, orbital lymphoma, orbital neurofibroma, Pott’s puffy tumor and other sinus infection

IV. Diagnosis and Discussion - Job’s Syndrome, or HIES, is a rare, inherited disease marked by high levels of immunoglobulin E (IgE), an antibody that triggers immune responses against parasites, infections and noninfectious allergens -Patients with HIES often have multi-system ailments involving skin, bones, or lungs and present with recurrent, dramatic skin infections -The exudative skin lesions were classic for impetigo ecthyma. The final ocular diagnosis was impetigo ecythma induced preseptal cellulitis -Orbital CT scan was imperative to rule out orbital cellulitis due to the severe ocular presentation and risk for morbidity/mortality

V. Treatment, management -Impetigo lesions are typically caused by staphylococcus aureus or streptococcus pyogenes, however, they can also be caused by methicillin resistant staphylococcus aureus (MRSA) -Differential diagnosis and treatment of the offending agent is critical -The patient was admitted to the hospital -A broad spectrum approach was taken until cultures were returned -Initial treatment was intravenous (IV) Cefazolin, then switched to IV Vancomycin, topical Mupirocin for facial lesions, and oral Clindamycin -The patient was also treated with Moxifloxacin ophthalmic solution TID and artificial tears prn -The preseptal cellulitis resolved over multiple follow up visits, and the patient was switched from IV Vancomycin to Doxycycline PO BID for a total antibiotic treatment of 14 days.

VI. Conclusion -Patients with HIES are more prone to contracting impetigo, a contagious exudative skin infection, that can spread to other parts of the body to include ocular involvement such as preseptal cellulitis -Although preseptal cellulitis may not be a serious condition, it can present concurrently with or progress to orbital cellulitis, which can be vision and life threatening. In these cases hospital admission is warranted.