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Sweet syndrome is an inflammatory condition characterized by the abrupt development of erythematous plaques accompanied by fever and neutrophilia. Different clinical presentations of this disorder have been described1 in the literature. We read with interest a recent report in JAMA Dermatology2 describing a new clinical variant of Sweet syndrome, namely, giant cellulitis-like Sweet syndrome. Clinically these patients develop large, red erythematous plaques that recur and often are misdiagnosed as cellulitis but are not responsive to systemic antibiotic therapy. However, these plaques respond to systemic corticosteroid therapy.
Recurrence of Sweet syndrome is common, and second-line treatments include colchicine, indomethacin, clofazimine, cyclosporine, and dapsone. We report a case in which dapsone was an effective treatment for recurrence of giant cellulitis-like Sweet syndrome.
Report of a Case
A woman in her 60s developed recurrent fever (temperature, 39°C) and a large erythematous plaque localized to the right thigh and flank. The diameter of the lesion was more than 50 cm (Figure 1). Laboratory examinations showed normal white blood cell counts, neutrophilia (white blood cells, 10 300/μL, and neutrophils, 8755/μL; to convert both values to ×109/L, multiply by 0.001), and negative tissue and blood bacterial cultures. The patient received levofloxacin and vancomycin with minimal response. The lesions resolved spontaneously; however, the patient experienced similar episodes several times within 3 years. Several skin biopsies of the lesions showed a superficial and middermal perivascular and interstitial inflammatory infiltrate consisting of lymphocytes and numerous neutrophils. Papillary dermal edema was present. No evidence of leukocytoclastic vasculitis or epidermal changes was seen. Tissue cultures failed to grow pathogenic bacteria, fungus, or atypical mycobacteria. Results of direct immunofluorescence studies were negative. Workup failed to show underlying solid or lymphoreticular cancer, rheumatoid arthritis, or inflammatory bowel disease. In the context of the clinical evolution and lack of response to broad-spectrum antibiotics, the diagnosis of an atypical form of Sweet syndrome was postulated. The patient was initially given colchicine (0.6 mg twice daily) and oral and intramuscular prednisone with response but subsequent relapse.3 In the context of the clinical findings and failure of the described therapeutic strategy, we discontinued colchicine therapy and started treatment with dapsone, 100 mg/d, and with oral prednisone, 20 mg/d.4 The patient’s lesions resolved with some postinflammatory pigment alterations (Figure 2). Prednisone therapy was tapered to discontinuation for 3 weeks. The patient continued to receive dapsone, 100 mg/d, without recurrence 10 months after initial presentation.
Large erythematous/edematous plaque over the patient’s thigh and flank.
The lesion resolved with some postinflammatory pigment alterations.
Neutrophilic dermatoses comprise a wide spectrum of inflammatory diseases with overlapping features characterized histologically by the presence of an aseptic neutrophilic infiltrate in the epidermis, dermis, and/or hypodermis. These dermatoses are often associated with systemic inflammatory and neoplastic disorders.2 Sweet syndrome includes variants as well as classic presentation, the cancer-associated presentation, and the drug-induced presentation.1
Our patient had severe, relapsing giant inflammatory lesions ranging from erythematous, slightly edematous, and infiltrated lesions to painful indurated plaques. The initial diagnosis was infectious cellulitis; however, antibiotic therapy failed to control the condition.
Standard treatment of Sweet syndrome is the administration of corticosteroids and, in relapsing or recurring lesions, corticosteroid-sparing agents may be required. Dapsone is one treatment for Sweet syndrome both as monotherapy or adjunctive to corticosteroid therapy.1,5 We wanted to bring attention to this newly described variant of Sweet syndrome as well as the safe and efficacious therapy of this entity with dapsone.
Corresponding Author: Hideki Koketsu, MD, Florida Academic Dermatology Centers, 1400 NW 12th Ave, Ste 4, Miami, FL 33136 (email@example.com).
Published Online: February 26, 2014. doi:10.1001/jamadermatol.2013.7814.
Author Contributions: Drs Koketsu and Ricotti had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.
Study concept and design: Koketsu, Ricotti.
Acquisition of data: All authors.
Analysis and interpretation of data: Koketsu, Ricotti.
Drafting of the manuscript: Koketsu, Ricotti.
Critical revision of the manuscript for important intellectual content: Koketsu, Ricotti.
Study supervision: All authors.
Conflict of Interest Disclosures: Dr Kerdel has received honoraria from AbbVie, Amgen, Astellas, Galderma, Janssen, LEO Pharma, and Medicis; has been a member of the speakers’ bureaus of AbbVie, Amgen, Astellas, Galderma, Janssen, LEO Pharma, and Medicis; and has received grants from AbbVie, Amgen, Jansen, and Pfizer. No other disclosures were reported.
Koketsu H, Ricotti C, Kerdel FA. Treatment of Giant Cellulitis-like Sweet Syndrome With Dapsone. JAMA Dermatol. 2014;150(4):457–459. doi:10.1001/jamadermatol.2013.7814
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