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Sweet's syndrome in a patient with Crohn's disease: a case report
© Mustafa and Lavizzo; licensee BioMed Central Ltd. 2008
Received: 06 August 2007
Accepted: 28 June 2008
Published: 28 June 2008
Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, has been associated with malignancy, autoimmune disease and collagen vascular disease. The association of Crohn's disease and Sweet's syndrome is rare. We report a case of Sweet's syndrome in a patient with Crohn's disease.
A 63-year-old man with a history of Crohn's disease presented with one-week duration of abdominal pain, diarrhea and hematochezia. He also noticed eruption of painful skin rashes all over his body at the same time. Colonoscopy and esophagogastroduodenoscopy (EGD) showed inflammation involving different parts of the gastrointestinal tract consistent with Crohn's disease. Punch biopsy of the skin lesion was consistent with Sweet's syndrome, which has a rare association with Crohn's disease.
Crohn's disease should be excluded in patients presenting with Sweet's syndrome and diarrhea. Alternatively, Sweet's syndrome should be considered as a diagnosis when a patient with Crohn's disease develops skin lesions.
Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, has rarely been associated with Crohn's disease. We report a case of Sweet's syndrome in a patient with Crohn's disease.
Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, was first described by Robert Douglas Sweet in 1964 . Sweet's syndrome is characterized by fever, neutrophilia, cutaneous eruptions consisting of erythematous papules and plaques, and a dermal nonvasculitic neutrophilic infiltration on skin biopsy [2, 3]. These plaques are painful but nonpruritic . Other skin manifestations such as pustules, vesicles, purpura, ulcers and hemorrhagic lesions have been described . Seventy-five percent of patients have some prodromal illness, most commonly an upper respiratory tract infection .
Common complications of Sweet's syndrome include arthralgia, arthritis, conjunctivitis, iridocyclitis, and rarely involvement of the central nervous system . Sweet's syndrome is more common in females with a female to male ratio of 3.7:1, with the mean age of 52 years .
Causes of Sweet's syndrome
Hematopeitic: myelodysplastic syndromes and acute myeloid leukemia, hairy cell leukemia, B and T cell lymphoma, agnogenic myeloid metaplasia
Solid tumors: breast, testicular, prostate, ovarian, vaginal squamous cell, genitourinary and gastrointestinal malignancies
Chronic active hepatitis, cytomegalovirus, human immunodeficiency virus
Streptococcus, mycobacterium, yersinia, typhus, salmonella
Autoimmune and collagen vascular diseases
Rheumatoid arthritis, systemic lupus erythematosus, mixed connective tissue disease, hashimoto thyroiditis, Sjogren's disease, behcet's disease
Furosemide, hydralazine, lithium, oral contraceptive pills, trimethoprim- sulfamethoxazole, minocycline and imatinib mesylate
Inflammatory bowel disease
Ulcerative Colitis, Crohn's disease
Subacute necrotizing lymhadenitis
Only a few cases of Sweet's syndrome associated with Crohn's disease have been reported in the literature . There is a higher incidence of colonic involvement and extraintestinal features in these patients. The skin lesions have been observed in patients with active Crohn's disease, but sometimes it can precede the onset of intestinal symptoms. It appears that the syndrome is not initiated by the underlying disease but rather shares with it a concurrent pathogenic mechanism.
The pathogenesis of Sweet's syndrome is poorly understood. Cytokines, such as granulocyte colony stimulating factor (G-CSF), interleukin (IL)-1, IL-6, or IL-8, if deposited in the dermis, may be responsible for the immunopathologic and clinical manifestations of Sweet's syndrome. The fact that Sweet's syndrome can occur after G-
CSF treatment shows that IL-1, which is produced by acute myelocytic leukemia (AML) cells and stimulates the G-CSF gene, plays a role in the pathogenesis of Sweet's syndrome .
For a definitive diagnosis of Sweet's syndrome, both major and two minor criteria should be met. The two major criteria are 1) abrupt onset of painful erythematous plaques or nodules occasionally with vesicles, pustules, or bullae and, 2) neutophilic infiltration in the dermis without leukocytoclastic vasculitis. The minor criteria are 1) skin lesions preceded by a nonspecific respiratory or gastrointestinal tract infection, vaccination or associated with inflammatory diseases such as autoimmune disorders, infections, hemoproliferative disorders, solid malignant tumors or pregnancy, 2) accompanied by periods of general malaise and fever (> 38°C), 3) laboratory values during onset: ESR > 20 mm, C reactive protein positive, segmented neutrophils >70% in peripheral blood smear, leukocytosis > 8000 (3 of 4 of these values are necessary), and 4) excellent response to treatment with systemic corticosteroids or potassium iodide [1, 8].
Sweet's syndrome is one of the groups of neutrophilic dermatoses that include pyoderma gangrenosum and whose association with ulcerative colitis and Crohn's disease is well established. Sweet's syndrome can be distinguished from pyoderma gangrenosum by the absence of vasculitis and lack of dermal necrosis, but histological features may occasionally overlap. The abrupt tendency for Sweet's syndrome to form multiple eruptions on the upper half of the body and the lack of ulceration also distinguishes the rash from pyoderma gangrenosum. However, the two conditions can occur in the same patient, as may other neutrophilic dermatosis, vesiculopapular eruptions or other cutaneous features of inflammatory bowel disease such as erythema nodosum or polyarthritis. The simultaneous occurrence of different rashes in the same person can be viewed as the dermatological expression of a neutrophilic reaction to a common stimulus .
Sweet's syndrome, if left untreated, usually heals within six to eight weeks .
Prednisone at an initial dose of 40–60 mg per day, with gradual tapering off over four to six weeks, is the standard treatment for Sweet's syndrome [3, 5]. Relapses are common if steroids are tapered too quickly. In recurrent disease, therapy with colchicine, potassium iodide, dapsone, doxycycline, indomethacin, clofazimine, isotretinoin and cyclosporine have all been described [1, 5].
Potassium iodide administered orally as 300 mg enteric-coated tablets, 3 times each day, for a daily dose of 900 mg, or as a saturated solution of potassium iodide (Lugol's solution), beginning at a dose of 3 drops 3 times each day (9 drops/day = 450 mg per day) and increasing by 1 drop 3 times per day, typically to a final dose of 21 drops/day (1050 mg) to 30 drops/day (1500 mg), usually results in resolution of fever and other symptoms within 1 to 2 days and resolution of skin lesions within 3 to 5 days of initiation of therapy. Vasculitis and hypothyroidism are potential adverse effects of potassium iodide .
Sweet's syndrome should be considered an extraintestinal manifestation of Crohn's disease, and should be differentiated from other more frequent inflammatory diseases that accompany Crohn's disease, like erythema nodusum, pyoderma gangrenosum and leukocytoclastic vasculitis. Awareness of this association may guide appropriate diagnostic procedures and therapy.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
Dr Niveditha Reddy MD.
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