A Case Report of Necrotizing Neutrophilic Dermatosis: A Sheep in Wolf's Clothing

Neutrophilic dermatosis (ND) is a category of diseases characterized by trauma-induced, autoinflammatory cutaneous eruption. Comorbid systemic disease is common with a predilection for malignancy, inflammatory bowel disease, and rheumatologic disease. Rarely, it can manifest with aseptic shock, an entity referred to as necrotizing neutrophilic dermatosis (NND). NND may occur in the postoperative setting and is often misdiagnosed as a necrotizing soft tissue infection. Unfortunately, the treatment for a necrotizing soft tissue infection, namely, wide debridement, is often detrimental in the setting of NND. We present the case of a woman with underlying myelodysplastic syndrome who developed episodic postoperative hemodynamic collapse followed by delayed necrotic peristomal ulceration following colonic diversion for complicated diverticulitis. Infectious workup and operative re-exploration were unrevealing. Pathologic assessment of affected skin tissue showed changes consistent with ND, ultimately leading to the diagnosis of NND. Her clinical course dramatically improved with the initiation of immunosuppressive therapy. The mimicry of NND to a potentially lethal necrotizing soft-tissue infection creates a grave diagnostic dilemma in the postoperative period. A general lack of knowledge of NND among non-dermatologic specialists produces an opportunity for misdiagnosis and inappropriate surgical interventions, namely, serial debridement. Several clinical cues may aid in the earlier recognition of NND. The cornerstone of treatment involves systemic corticosteroid therapy with adjunctive therapy for refractory cases. NND must be considered in the differential diagnosis of necrotizing soft tissue infection as early recognition may result in the avoidance of deleterious surgical interventions.