P081 - NEUTROPHILIC PUSTULOSIS OF ULCERATIVE COLITIS: A SPECTRUM OF EXTRAINTESTINAL DISEASE
(Room Poster Hall)
19 Jan 18
5:30 PM
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7:00 PM
Tracks:
Clinical and Research Challenges
BACKGROUND: The pustular eruption of ulcerative colitis, also known as neutrophilic pustulosis, is a poorly defined, yet well-described extraintestinal manifestation of ulcerative colitis (UC), occurring in 1-6% of cases. METHODS: We report a case of a 23-year old female with a history of UC diagnosed at age 13, neutrophilic pustulosis at age 17, and 2 months of bloody diarrhea and abdominal pain who presented to the hospital with a diffuse pustular eruption concerning for neutrophilic pustulosis. One such purulent lesion on her breast rapidly ulcerated and expanded over days, concerning for pyoderma gangrenosum (PG). We conducted a review of the literature for cases of neutrophilic pustulosis. RESULTS: Attempts to define the pustular eruption of UC are inconsistent, with some calling it a distinct entity, some a variant of Sweet’s syndrome, and some a bullous or pustular variant of PG. This distinction may be arbitrary, as all of these conditions fall into the spectrum of neutrophilic dermatosis, a group of autoinflammatory conditions associated with systemic disease, especially inflammatory bowel disease, and characterized by neutrophilic infiltration of skin and rarely of deeper organs. CONCLUSIONS: As highlighted by this case, the clinical and morphologic picture of the specific neutrophilic dermatosis is not always clear and there can be significant overlap between the syndromes, making exact diagnosis challenging. A paradigm shift is instead needed to address these diseases as a spectrum of extraintestinal manifestations of IBD. Prompt recognition of the variety of the diseases that fall on this spectrum can allow for rapid initiation of appropriate therapy.