Rare Association of Crohnʼs Disease and Sweetʼs Syndrome: A Case Report

Abstract

Purpose: A 43-year-old Caucasian female with a history of hypothyroidism, anxiety and a recent diagnosis of Crohn's Disease (CD), presented with four days of progressive itchy rash on both arms, fever, itchy eyes, arthralgias, and URI symptoms. Four weeks prior to this presentation, the patient was admitted with diarrhea. Work-up, which included a colonoscopy, revealed changes consistent with CD in the terminal ileum and the ileocecal valve. Patient was started on a combination of Entocort and azathioprine. Slowly, her CD symptoms slightly improved. Ten days later, the azathioprine dose was increased to 75 mg per day. One week following this dose adjustment, a series of new symptoms emerged. Patient noted tender red bumps that were multiplied in number and increased in size, covering her entire right upper arm and a few small red spots on the left arm. Mono Spot and Rapid Strep were both negative at that point. Patient was started on Hydroxyzine and Valacyclovir. Symptoms did not improve. On the morning of admission, patient had a temperature of 102.1 F. Her rash now covered her upper arms bilaterally. She had also developed a few small red spots on both her thighs. Exam was significant for very dramatic swollen lymph nodes on the anterior neck, multiple edematous redpurple color papules and plaques measuring 0.5-2 cm on bilateral upper arms and erythematous palpebral conjunctiva. Many of these had very edematous centers and almost looked vesicular and pustular in appearance. She also had scarce scattered 2 mm pinpoint erythematous papules on legs, trunk and back. Her lab results were remarkable for neutrophilia, elevated CRP and ESR. Subsequently, a punch biopsy was obtained. The azathioprine was discontinued with the suspicion that the lesion was secondary to medication side effects. The biopsy demonstrated a spongiotic epidermis with focal basal vacuolar changes and papillary edema. There was a dense diffuse infiltrate of mature neutrophils in the upper half of the dermis. Leukocytoclasis with formation of nuclear ducts was present but there was no evidence of vasculitis. The deep dermis and subcutaneous tissue were free of involvement. These findings are consistent with neutrophilic dermatosis and Sweet's Syndrome (SS). Prednisone 40 mg po daily was started for treatment of her SS. Patient's skin rash started to resolve with steroids and her conjunctivitis also improved. Patient was discharged on prednisone. Follow-up at five weeks post disposition confirmed that the patient was clear of any new welts and ocular symptoms. We recommend that SS be considered in the differential diagnosis in CD patients who develop skin lesions especially if the patients are started on drugs such as azathioprine.