Extensive Cellulitic Infection and Tissue Necrosis in a Patient With Hyper IgE Syndrome: Surgical Management of a Rare Immunodeficiency.

Hyper IgE syndrome (HIES) is a rare primary immunodeficiency that is characterized by elevated serum IgE levels, recurrent sinopulmonary infections, and chronic eczema, among other symptoms. Though reports on patients with HIES exist, they primarily focus on the clinical features, diagnosis, and management of HIES without detailing surgical interventions. Here, we present the surgical management of an extensive cellulitic infection that developed into skin necrosis in an HIES patient with a history of polysubstance abuse. The patient had a diagnosis of Job's syndrome, the autosomal dominant form of HIES. In this case, severe infection resulting from immunodeficiency led to cellulitis and full-thickness tissue loss in the left upper limb. Due to the extent of infection and necrosis, multiple debridements and upper extremity reconstructions were necessary for limb preservation. The 625 cm² wound, which involved the left upper extremity and crossed the elbow joint, was managed with initial debridement followed by a period of local wound care to allow for clearance of infection. We then performed reconstruction in a staged fashion with dermal substitute (Integra, Integra LifeSciences Holdings Corporation, Princeton, NJ) placement and split-thickness skin grafting (STSG). In this case, we demonstrate that the extensive soft tissue infections that may be found in HIES patients can be successfully managed with skin grafting and dermal substitutes.