Sweet Syndrome Accompanying Inflammatory Dehydrogenase Deficiency and Its Association Bowel Disease in a Child

Abstract

Acute neutrophilic dermatosis, first described in 1964 byRobert Douglas Sweet, is characterized by sudden onset fever,neutrophilic leukocytosis, and well demarcated erythematouspapules, nodules, and plaques with dense neutrophilic infiltrateson histologic evaluation.Here is a report of a 7-year-old girl who presented withhigh grade fever, and discrete erythematous papular skin eruptions,which gradually increased in number and involved theface, trunk, extremities, palms, soles, hard palate, and palataltonsils. The skin eruptions evolved to pustules and after coalescingcaused large crusted plaques, with mild tenderness butwithout any pruritus. White blood cells were 36900/ml with92% neutrophils. Skin biopsy test was compatible with acutefebrile neutrophilic dermatosis, so prednisolone (1 mg/kg/day)was started that led to a rapid defervescence and significantimprovement of dermatosis. After a few days, the patient presentedwith fever and arthritis of right elbow, both ankles, andwrists, so she was re-admitted. She also developed bloody diarrheaduring the hospital stay. Colonoscopy and intestinalbiopsy were performed, which confirmed the diagnosis of ulcerativecolitis. Prednisolone, sulfasalazine, and naproxenwere prescribed. The fever and diarrhea stopped after a fewdays and joint swelling decreased. She was discharged 2weeks after the admission with a rather good general condition.Inflammatory bowel disease can be one of the severalconditions accompanying sweet syndrome.

Keywords

• Sweet syndrome
• acute neutrophilic dermatosis
• inflammatory bowel disease