Histiocytoid Sweet syndrome is infiltrated predominantly by M2-like macrophages - 13/12/14
Abstract |
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.
Le texte complet de cet article est disponible en PDF.Key words : histiocytoid Sweet syndrome, M-2 like macrophages, myelodysplastic syndrome, Sweet syndrome
Abbreviations used : HIER, HSS, MDS, MPO, SS
Plan
Drs Peroni and Colato equally contributed to the manuscript. |
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Funding sources: None. |
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Conflicts of interest: None declared. |
Vol 72 - N° 1
P. 131-139 - janvier 2015 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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