Histiocytoid Sweet syndrome is infiltrated predominantly by M2-like macrophages

作者：Peroni Anna; Colato Chiara; Schena Donatella; Rongioletti Franco; Girolomoni Giampiero^*

来源：Journal of the American Academy of Dermatology, 2015, 72(1): 131-139.

DOI：10.1016/j.jaad.2014.09.025

• histiocytoid Sweet syndrome
• M-2 like macrophages
• myelodysplastic syndrome
• Sweet syndrome

Background: Histiocytoid Sweet syndrome (HSS) is a rare variant of Sweet syndrome (SS). The nature of histiocytoid cells is still uncertain.
Objective: We sought to offer a comprehensive overview on clinical features of HSS and further information on immunohistochemical phenotype of the infiltrate.
Methods: The clinical, histologic, and immunohistochemical features of 12 of our patients with HSS and all cases retrieved through a PubMed search were analyzed.
Results: Lesions consisted of erythematous-violaceous papules and plaques, randomly distributed mostly on the trunk and the limbs. Three patients had myelodysplastic syndrome and 1 had a monoclonal gammopathy. The infiltrate was mainly composed of CD68(+) CD163(+) myeloperoxidase(+) myeloid cell nuclear differentiation antigen (+)CD117(-) CD15(-) CD34(-), a phenotype suggestive of M2-like macrophages. A few mature neutrophils and lymphocytes were also present. Review of all HSS cases showed no sex predominance and no extracutaneous infiltrates; inconstant presence of fever and blood neutrophilia; association with hematologic or solid neoplasms (26%), autoimmune conditions (12%), and infectious diseases (10%); and good response to steroid treatment, with rare relapses or recurrences.
Limitations: The study includes a limited case series. The pathogenesis of the disease remains to be clarified.
Conclusions: HSS lesions are infiltrated mostly by M2-like macrophages. The clinical features present more similarities than differences with SS.

• 出版日期2015-1