Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a systemic disease involving nodal and extranodal tissues. We report a 48-year-old female with recurrent nasal obstruction due to polypoidal masses involving the nasal sinuses, turbinates and septum bilaterally, and lumps in the right infra-orbital region and region of the right lacrimal sac. ...

Fever not explained by infection may occur in patients with malignant lymphoma presumably caused by a release of endogenous pyrogen. Although pyrogen has been found in some tumors with a mixed cell population, production of endogenous pyrogen by the neoplastic cells has not been demonstrated. This report documents the apparently spontaneous synthesis and release of such pyrogen by two human tum...

Xanthoma disseminatum is a rare normolipemic histiocytic disorder of non-Langerhans cell origin. It is a chronic systemic disease with a benign course, characterized by disseminated, yellow-orange-colored papules on the face, flexures, and mucosal membranes. We report 3 patients with xanthoma disseminatum, who presented primarily with central nervous system disease and a multitude of imaging fi...

Nodular histiocytic/mesothelial hyperplasia (NHMH) is a very rare condition. It generally thought to be associated with repeated mechanical or chemical stimulation. This study reported NHMH papillary growth pattern in the ovary following high-intensity focused ultrasound (HIFU) treatment for uterine leiomyoma and adenomyosis. A 48-year-old female, who had history of undergoing HIFU 7 times aden...

The etiology of cervical lymphadenopathy is multifactorial including infections and tumoral lesions. Histiocytic necrotising lymphadenitis or Kikuchi-Fujimoto's disease is a rare cause of cervical lymphadenopathy which does not have specific clinical signs. We report herewith a case of Kikuchi's disease (KD) diagnosed by histopathology.

Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing lymphadenitis, is rare condition that usually presents with lymphadenitis and fever. KFD has been associated with many infectious disease processes, predominantly viral. Association with systemic inflammatory processes has been described. Here we present a case of KFD with the rare ocular manifestation of bilateral anterior uve...

Immunoglobulin-G4 (IgG4)-related disease (IgG4RD) is a fibro-inflammatory disorder characterized by tissue-infiltrating IgG4 plasma cells, and, often, high serum IgG4. Several autoimmune, infectious, or proliferative conditions mimic IgG4RD. Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, characterized by foamy histiocytic infiltration, fibrosis, and chronic inflammat...

Rosai-Dorfman disease is a rare, idiopathic, benign, and self-limiting histiocytic proliferative disorder. A 26-year-old man presented with a single massive cutaneous nodule (reaching 30 cm in diameter) on the left shoulder and back for 15 months. The routine hematological and biochemical tests were normal. Magnetic resonance scanning showed the lesion involved the skin, subcutaneous tissue, an...

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy, SHML) is a rare, non-hereditary, benign histiocytic proliferative disorder mainly affecting young people with common clinical characteristics such as painless bilateral cervical lymphadenopathy, fever, leukocytosis and polyclonal gammopathy. Extranodal manifestations have been reported in 28-43% of cases. Eye involvement i...