Facial Impetigo and Preseptal Cellulitis Associated with Job's Syndrome, A
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.
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