P-019 Sweetʼs Syndrome as an Extraintestinal Ulcerative Retocolitis Manifestation

Abstract

Inflammatory Bowel Diseases (IBD) are associated with a variety of extraintestinal manifestations and can virtually affect every organ. The incidence of cutaneous manifestations occurs in the range from 2% to 34% of the cases and are presented as erythema nodosum, pyoderma gangrenosum and Sweet's Syndrome (SS). The Sweet's syndrome (SS) is a rare form with strong predilection for females (87%), colonic involvement (100%) and other extra-intestinal findings (77%). It also has association with the disease activity from 67% to 80% of the cases. It is characterized by the appearance of erythema, erythematous papules or painful but not itchy nodules with preferential involvement of the face, neck and upper limbs, accompanied by fever, neutrophilia and neutrophilic infiltrate in the upper dermis. Diagnostic criteria are clinical and histopathological as proposed by Su and Liu in 1987 which remain valid today. The most effective treatment is immunosuppressive therapy, most commonly with systemic corticosteroids at doses ranging from 40 and 80 mg/day of Prednisone with good efficacy. Case report and literature review. This current report documents the rare extra-intestinal manifestation developed by a male patient of 47 years old, a former smoker, accompanied at the Inflammatory Bowel Diseases Clinic of the Faculty of Medicine of Botucatu (FMB) for UC—pancolitis, of difficult control. The pathology report showed colitis in intense activity with microabscesses and crypts, and a merge of them. Cutaneous effects began 10 months after diagnosis, with the appearance of erythematous papules on target with central umbilication, some of them resembling pustules and tendency to central ulceration on the face, trunk and limbs for 2 days (Fig. 1). She also reported a peak fever and was admitted to hospital with leukocytosis, at the expense of neutrophilia and elevated inflammatory activity tests. A performed skin biopsy showed an inflammatory infiltrate of predominantly neutrophilic nature in the dermis without leukocytoclastic vasculitis. With a confirmed diagnosis of Sweet's Syndrome she started steroids treatment (40 mg/day of Prednisone) and a significant improvement of the skin condition was obtained after a month of onset. After 3 months of therapy at weaning of steroids (Prednisone 20 mg/day), there was only a slight erythema and some secondary acneiform eruptions to the medication with no additional active lesions (Fig. 2). The patient remains under follow-up with no recurrence of the condition. The completion of well documented and internationally accepted clinical criteria was the real milestone to confirm the diagnosis of patient's cutaneous condition which was only ratified by the excellent response to treatment with corticosteroids. Finally, when IBD is mentioned the importance of a detailed clinical follow-up is recognized regardless of the organ and multi-professionalism involved for clinical suspicion of rare manifestations as this one described in this patient.