Introduction: Non-Langerhans cell histiocytoses (non-LCH) are a group of rare diseases with varied clinical manifestations and overlapping features seen among the subtypes. Here, we present a case of Rosai-Dorfman disease with features of necrobiotic xanthogranuloma. Case: A 45-year-old female presented with a 10-year history of an enlarging neck mass with normal overlying skin accompanied by dysphagia and multiple asymptomatic pink to yellowish-brown papules, nodules, and plaques on the face, trunk and extremities. Biopsies of a skin nodule and plaque revealed granulomatous dermal infiltrates (lymphocytes, foamy histiocytes, and Touton giant cells), emperipolesis and areas of necrosis. CD1A and Fite-Faraco staining showed negative results while CD68 and S100 positively stained the tissues of interest. Histopathology of the neck mass paralleled these findings in addition to being negative for lymphoid markers. Patient had monoclonal gammopathy and thyromegaly with enlarged cervical lymph nodes on further tests and imaging. Intralesional and systemic steroids were given which led to flattening of skin lesions and improvement in dysphagia, respectively. Conclusion Diagnosis and classification of a particular type of non-LCH may be difficult due to similarities across its subtypes. Hence, it is our belief that these diseases may occur on a spectrum. Treatment involves a multidisciplinary approach for the best possible care.
Histiocytosis ; Histiocytosis, Sinus ; Necrobiotic Xanthogranuloma

No abstract available.
Histiocytosis, Sinus* ; Lip*

No abstract available.
Histiocytosis, Sinus* ; Lymphatic Diseases*

Histiocytosis, Sinus ; diagnosis ; Humans

Histiocytosis, Sinus ; Humans

OBJECTIVES: To present a case of Rosai-Dorfman disease in an individual with a 14-year history of recurrent nasal polyposis and discuss its clinical presentation, physical examination, radiologic findings, histopathologic characteristics and available treatment. METHODS: Design: Case Report Setting: Tertiary Government Hospital Patient: One RESULTS: A 26-year-old Filipino diagnosed and repeatedly treated medically and surgically for recurrent nasal polyposis underwent repeat endoscopic sinus surgery. A histopathologic impression of Rosai-Dorfman disease was confirmed by positive S-100 and CD1a negative immunochemistry in conjunction with the morphologic findings. CONCLUSION: Rosai-Dorfman disease is a rare entity which should be considered when dealing with recurrent nasal polyposis that is intractable to initial medical and surgical therapies. Histopathologic findings of emperipolesis and immunohistochemical S-100 stains play a key role in the diagnosis but there is yet no definite treatment for this disease.
Human ; Adult ; Histiocytosis, Sinus ; Emperipolesis

No abstract available.
Foramen Magnum* ; Histiocytosis, Sinus* ; Spinal Cord Compression*

Humans ; Histiocytosis, Sinus/diagnosis* ; Diagnosis, Differential ; Lung

Rosai-Dorfman disease also known as sinus histiocytosis with massive lymphadenopathy is a benign, rare systemic disease characterized by a histiocyte proliferation which presents with lymphadenopathy. We report a case of a 4-year-old girl who presented with recurrent cervical lymphadenopathy with tenderness, without any other symptoms. After 1 month of medical treatment, her lymphadenopathy still remained, so we performed complete excision and biopsy. She was diagnosed on cytology as a case of Rosai-Dorfman disease. She responded well to become asymptomatic without recurrence by 1 month.
Biopsy ; Child ; Child, Preschool* ; Female* ; Histiocytes ; Histiocytosis ; Histiocytosis, Sinus* ; Humans ; Lymphatic Diseases ; Recurrence

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder of lymph node and extranodal site. Bone involvement is very rare. We report a case of extranodal RDD of the tibia in 32-year old male. The patient presented with pain with no evidence of lymphadenopathy. Clinico-radiologic diagnosis was metastatic carcinoma or Langerhans cell histiocytosis, but, histopathologic examination confirmed the diagnosis with RDD. We performed curettage on the osteolytic lesion of tibia. In South Korea, there was no report about RDD of the extremity and we want to report this case with review of the literature.
Curettage ; Diagnosis ; Extremities ; Histiocytosis, Langerhans-Cell ; Histiocytosis, Sinus* ; Humans ; Korea ; Lymph Nodes ; Lymphatic Diseases ; Male ; Tibia*