Subcutaneous Sweet syndrome in the setting of myeloid disorders: A case series and review of the literature

• Published in: Journal of the American Academy of Dermatology 2013-06, Vol.68 (6), p.1006-1015
• Main Authors: Chan, May P., MD, Duncan, Lyn M., MD, Nazarian, Rosalynn M., MD
• Format: Article
• Language: English
• Subjects: Adult; Dermatology; Female; Humans; Leukemia, Myeloid, Acute - complications; Male; Middle Aged; myelodysplastic syndrome; Myelodysplastic Syndromes - complications; myeloid leukemia; neutrophilic dermatosis; neutrophilic panniculitis; Pancytopenia - complications; Panniculitis - complications; Panniculitis - pathology; subcutaneous; Sweet syndrome; Sweet Syndrome - complications
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2012.12.954

Background The association of neutrophilic dermatoses with myeloid disorders is well known, but neutrophilic panniculitis in the same setting has only been reported infrequently. Objective We aimed to better characterize these lesions clinically and histologically, and to provide a comprehensive differential diagnosis and appropriate diagnostic approach. Methods The pathology archives were searched for cases of neutrophilic panniculitis in patients with myeloid disorders. Clinical history and histologic features were studied. Similar cases in the English-language literature were reviewed. Results Five biopsy specimens from 4 patients and 11 previous case reports were identified. All patients presented with erythematous tender nodules typically involving the extremities. Most were accompanied by fevers, and resolved either spontaneously or with steroids. Histologically, the subcutaneous neutrophilic infiltrates demonstrated lobular or, less frequently, septal patterns with minimal dermal involvement. Leukocytoclasis, fat necrosis, reactive stromal fibroblasts, and mild reticular dermal edema were each seen in at least 3 of our 5 biopsy specimens. No myeloid blasts, vascular changes, or non-Miescher granulomas were observed. No micro-organisms were identified in the skin nodules. Limitations This study is limited by the small number of cases. Conclusions Based on the fairly consistent clinical and histologic findings, neutrophilic panniculitis occurring in the setting of myeloid disorders is best classified as subcutaneous Sweet syndrome after exclusion of infectious panniculitis, id reaction, and leukemia cutis. These lesions may be associated with myeloid disorders, or induced by chemotherapy or other medications.