Xanthomatized Neutrophilic Dermatosis in a Patient With Myelodysplastic Syndrome

We present an original case report of a 58-year-old man with a history of histiocytoid Sweet and myelodysplastic syndrome who presented with a new onset of tender plaques on his oral commissures. A biopsy revealed a dense dermal neutrophilic infiltrate, leukocytoclasis without frank vasculitis, and, most notably, the presence of many xanthomatized cells. Clinical presentation and histologic features were most consistent with an acute neutrophilic dermatosis, which we believe to be a xanthomized variant of Sweet syndrome. Recent cases have described patients with similar clinical features and neutrophilic dermatosis in the setting of a normolipemic xanthomatosis. These cases were ultimately diagnosed as neutrophilic xanthoma, suggesting a possible histologic overlap between neutrophilic xanthoma and Sweet syndrome. Given these findings, we propose xanthomatized Sweet syndrome as a new histologic variant.