Giant cellulitis-like Sweet syndrome in the setting of autoimmune disease
Authors: Edidiong Celestine Ntuen Kaminska, Adaobi I Nwaneshiudu, Arlene Ruiz de Luzuriaga, Maria Tsoukas, Diana Bolotin
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: An unusual presentation of Sweet syndrome, giant cellulitis-like type, has recently been reported in the setting of malignancy and morbid obesity.1Surovy A.M. Pelivani N. Hegyi I. Buettiker U. Beltraminelli H. Borradori L. Giant cellulitis-like Sweet syndrome, a new variant of neutrophilic dermatosis.JAMA Dermatol. 2013; 149: 79-83Crossref PubMed Scopus (30) Google Scholar We report a case of giant cellulitis-like Sweet syndrome in a patient with autoimmune disease in the absence of malignancy. A 54-year-old woman with history of sicca syndrome, recurrent primary biliary cirrhosis (PBC) despite a liver transplant for PBC 16 years previously, and morbid obesity presented with a new red rash of at least 3 days' duration. She was initially hospitalized for Escherichia coli bacteremia (1 month before rash onset) that was treated with intravenous cefepime for 2 weeks; subsequent bacterial blood cultures were negative. Despite successful eradication of the bacteremia, chills and fevers associated with the new rash developed. Initially the rash was treated as cellulitis but did not respond to treatment with intravenous vancomycin. On examination (Fig 1), there were multiple large, scattered, erythematous, edematous, indurated papules and confluent plaques on her buttocks, extremities, and trunk; the eruption progressed to involve the head and neck. Punch biopsies of a papule and plaque on the patient's back revealed prominent papillary dermal edema with a mixed inflammatory cell infiltrate composed of abundant neutrophils intermixed with lymphocytes, histiocytes, and eosinophils (Fig 2), consistent with Sweet syndrome. Leukocytoclastic vasculitis was not noted. Serum blood counts and chemistry demonstrated a normocytic anemia with hemoglobin of 8.1 g/dL, normal white blood count of 4.1 K/μL (with a slightly increased neutrophil count of 77%), and increased creatinine of 1.7 mg/dL. Tissue cultures for bacteria and fungi were negative. The patient was treated with a prednisone taper over a course of 40 days. The lesions improved rapidly within 48 hours after initiation of treatment. Rare clinical presentations of Sweet syndrome include necrotizing fasciitis and cellulitic types,1Surovy A.M. Pelivani N. Hegyi I. Buettiker U. Beltraminelli H. Borradori L. Giant cellulitis-like Sweet syndrome, a new variant of neutrophilic dermatosis.JAMA Dermatol. 2013; 149: 79-83Crossref PubMed Scopus (30) Google Scholar, 2Kroshinsky D. Alloo A. Rothschild B. Cummins J. Tan J. Montecino R. et al.Necrotizing Sweet syndrome: a new variant of neutrophilic dermatosis mimicking necrotizing fasciitis.J Am Acad Dermatol. 2012; 67: 945-954Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar, 3Withrow R.A. Shenenberger D. Kalish V.B. Krivda S. Cellulitis unresponsive to antibiotics. Sweet's syndrome.J Fam Pract. 2011; 60: 149-151PubMed Google Scholar, 4Dinh H. Murugasu A. Gin D. Sweet's syndrome associated with cellulitis.Australas J Dermatol. 2007; 48: 105-109Crossref PubMed Scopus (12) Google Scholar, 5Crum N.F. Higginbottom P.A. Fehl F.C. Graham B.S. Sweet's syndrome masquerading as facial cellulitis.Cutis. 2003; 71: 469-472PubMed Google Scholar and do not respond to antibiotics.3Withrow R.A. Shenenberger D. Kalish V.B. Krivda S. Cellulitis unresponsive to antibiotics. Sweet's syndrome.J Fam Pract. 2011; 60: 149-151PubMed Google Scholar Widespread “giant cellulitis-like” Sweet syndrome, such as presented in this case, was recently reported in 3 patients.1Surovy A.M. Pelivani N. Hegyi I. Buettiker U. Beltraminelli H. Borradori L. Giant cellulitis-like Sweet syndrome, a new variant of neutrophilic dermatosis.JAMA Dermatol. 2013; 149: 79-83Crossref PubMed Scopus (30) Google Scholar All of the cases described obese patients with recurrent fever and widespread infiltrated plaques with bullous characteristics. Histopathology revealed classic findings of Sweet syndrome. Two patients were found to have an underlying malignancy (multiple myeloma or breast cancer); the other patient had no identified malignancy or autoimmune disease.Fig 2Giant cellulitis-like Sweet syndrome pathology. Prominent papillary dermal edema (top arrow) with abundant dermal neutrophils (bottom arrow).View Large Image Figure ViewerDownload Hi-res image Download (PPT) To our knowledge, an extensive cellulitic presentation of Sweet syndrome has only been described in the 3 patients mentioned here. Like in these cases, our patient had severe widespread eruption of giant cellulitis-like plaques and was morbidly obese. However, to date, no malignancies have been identified in our patient. Because Sweet syndrome may herald onset or recurrence of malignancy,1Surovy A.M. Pelivani N. Hegyi I. Buettiker U. Beltraminelli H. Borradori L. Giant cellulitis-like Sweet syndrome, a new variant of neutrophilic dermatosis.JAMA Dermatol. 2013; 149: 79-83Crossref PubMed Scopus (30) Google Scholar, 3Withrow R.A. Shenenberger D. Kalish V.B. Krivda S. Cellulitis unresponsive to antibiotics. Sweet's syndrome.J Fam Pract. 2011; 60: 149-151PubMed Google Scholar routine screenings and close follow-up are recommended. Our patient had classic Sweet syndrome in the setting of precedent bacteremia and inflammatory diseases (sicca syndrome and PBC), yet she presented with unusual giant cellulitis-like features. It is likely that giant cellulitis-like Sweet syndrome may be an uncommon clinical presentation for the different variants of Sweet syndrome and may not be restricted to the malignancy type.