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

Necrobiotic xanthogranuloma (NXG) is a rare multisystemic disease that manifests as cutaneous inflammatory lesions and has a strong association with paraproteinemia and an increased risk of plasma cell dyscrasias and other lymphoproliferative disorders. Its general skin manifestation is multiple, asymptomatic, indurated nodules or plaques with a yellowish hue which frequently affect the periorbital regions. This case is unusual in that cutaneous lesions developed only at the extremities without periorbital involvement, accompanied by multiple myeloma.
Extremities ; Lymphoproliferative Disorders ; Multiple Myeloma* ; Necrobiotic Xanthogranuloma* ; Paraproteinemias ; Skin Manifestations

Necrobiotic xanthogranuloma (NXG) is a rare, progressive, histiocytic disease characterized by destructive cutaneous lesions, a close association with paraproteinemia, multiple myeloma, hypertension, and multiple extracutaneous manifestations. Multiple indurated yellow-red plaques or nodules are commonly present in this disorder. The pathogenesis of NXG is poorly understood. Histopathologically, NXG features with hyaline necrosis or necrobiosis are present. We describe a patient with typical cutaneous and histologic findings of NXG with an associated monoclonal gammopathy.
Humans ; Hyalin ; Hypertension ; Multiple Myeloma ; Necrobiotic Disorders ; Necrobiotic Xanthogranuloma ; Necrosis ; Paraproteinemias

We report an unusual case of necrobiosis lipoidica with prominent cholesterol clefts in a 36-year-old man who had developed multiple, 0.5~5cm in diameter, round to oval-shaped erythematous plaques on both shins for over 3 years. Laboratory findings showed the following; cholesterol, 250mg/dl; triglyceride, 208mg/dl; blood sugar level fasting/2-h postprandial, 101/92mg/dl; and other findings were within normal limits or negative. There was no paraproteinemia. The biopsy specimen showed various lymphohistiocytic granuloma with many giants cell in the mid and deep dermis, and septal fibrosis in the subcutaneous tissue. There were also extensive areas of necrobiosis with prominent cholesterol clefts mimicking necrobiotic xanthogranuloma.
Adult ; Biopsy ; Blood Glucose ; Cholesterol* ; Dermis ; Fibrosis ; Granuloma ; Humans ; Necrobiosis Lipoidica* ; Necrobiotic Disorders* ; Necrobiotic Xanthogranuloma ; Paraproteinemias ; Subcutaneous Tissue ; Triglycerides

PURPOSE: To investigate the role of nitric oxide (NO) on the migration of cultured human Tenon's capsule PURPOSE: Necrobiotic xanthogranuloma is a very rare disease characterized by the presence of yellow subcutaneous skin lesions and systemic paraproteinemia. We report a case of post traumatic necrobiotic xanthograuloma that presented with a preseptal mass. CASE SUMMARY: A 31-year-old man presented with ptosis and a mass on the left superior orbit that developed after trauma 3 weeks prior to admission. A computed tomography scan showed a contrast-enhancing preseptal mass, and surgical exploration was performed. CONCLUSIONS: Histopathological examination revealed a tumor that had all the major features of necrobiotic xanthogranuloma, including fat necrosis with granulomatous inflammation, giant multinucleated cells, foamy cells, and several lymphoid follicles with germinal centers. Hematologic evaluation revealed polyclonal gammopathy.
Adult ; Fat Necrosis ; Germinal Center ; Humans ; Inflammation ; Necrobiotic Xanthogranuloma ; Nitric Oxide ; Orbit ; Paraproteinemias ; Rare Diseases ; Skin ; Tenon Capsule

Necrobiotic xanthogranulomatous reaction is a multiorgan, non-Langerhans cell histiocytosis with an unknown etiology. Occurrence in the salivary gland is extremely rare. We recently identified a case of necrobiotic xanthogranulomatous sialadenitis in a 73-year-old Korean woman who presented with a painless palpable lesion in the chin. There was no accompanying cutaneous lesion. Partial resection and subsequent wide excision with neck dissection were performed. Pathological examination showed a severe inflammatory lesion that included foamy macrophages centrally admixed with neutrophils, eosinophils, lymphocytes, plasma cells, and scattered giant cells, as well as necrobiosis. During the 12-month postoperative period, no grossly remarkable change in size was noted. Necrobiotic xanthogranulomatous inflammation may be preceded by or combined with hematologic malignancy. Although rare, clinicians and radiologists should be aware that an adhesive necrobiotic xanthogranuloma in the salivary gland may present with a mass-like lesion. Further evaluation for hematologic disease and close follow-up are needed when a pathologic diagnosis is made.
Adhesives ; Aged ; Chin ; Diagnosis ; Eosinophils ; Female ; Follow-Up Studies ; Giant Cells ; Hematologic Diseases ; Hematologic Neoplasms ; Histiocytosis ; Humans ; Inflammation ; Lymphocytes ; Macrophages ; Neck Dissection ; Necrobiotic Disorders ; Necrobiotic Xanthogranuloma ; Neutrophils ; Plasma Cells ; Postoperative Period ; Salivary Glands ; Sialadenitis ; Skin ; Submandibular Gland

We have recently experienced a case of xanthogranuloma in an adult, presenting as a round, pedunculated mass on the upper eyelid. Laboratory findings, including blood lipid substances such as cholesterol and triglycerides, were within normal limits. Furthermore, we could not find any extracutaneous manifestations. However, histopathologic findings revealed granulomatous infiltrates containing foamy cells, foreign body giant cells, Touton giant cells, as well as histiocytes and lymphocytes. Differential diagnosis with necrobiotic xanthogranuloma and Erdheim-Chester disease is essential when the lesion is observed in the periocular area, and additional systemic evaluation and regular follow-up examination are required.
Adult* ; Cholesterol ; Diagnosis, Differential ; Erdheim-Chester Disease ; Eyelids* ; Follow-Up Studies ; Giant Cells ; Giant Cells, Foreign-Body ; Histiocytes ; Humans ; Lymphocytes ; Necrobiotic Xanthogranuloma ; Triglycerides