The absence of feature of hemophagocytosis and criteria used for the diagnosis of HLH ruled out HLH, the absence of increased IgG4-positive plasma cell ruled out IgG4 disease, negativity for CD1a and Langerin ruled out Langerhans cell histiocytosis, histomorphology and clinical findings ruled out sinus histiocytosis, and histomorphology along with characteristic immunostain positivity for CD68 and S100 along with clinical findings ruled out various lymphomatous neoplasms involving the region. Here, CD207 is linked to hemophagocytic syndrome.