Traversing the spectrum of non-Langerhans Cell Histiocytosis: A case of Rosai-Dorfman Disease with features of Necrobiotic Xanthogranuloma

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.

Viejas enfermedades conocidas que afectan la órbita: Presentación de un caso clínico y revisión / Old known diseases affecting the orbit: Presentation of a clinical case and review

Presentamos el caso de un paciente de 56 años con antecedente de Mieloma Múltiple, que evoluciona con inflamación periorbitaria de ambos parpados, bilateral, de coloración amarillenta-violácea, ulcerada e indolora. Se planteó el diagnóstico diferencial con las enfermedades xantogranulomatosas orbitarias del adulto, en especial con su asociación con Mieloma Múltiple (MM). Al realizar biopsia de la lesión, se observan en la histología fragmentación de haces de colágeno dérmico y alrededor de vasos un material amorfo acelular eosinófilo pálido, rojo congo positivo y coloración verde manzana con luz polarizada, llegando al diagnóstico de Amiloidosis y Xantogranuloma Orbitario del Adulto.

We present the case of a 56-year-old patient with a history of Multiple Myeloma, who evolves with periorbital inflammation of both eyelids, bilateral, yellowish-violet, ulcerated and painless. The differential diagnosis with the adult orbital xanthogranulomatous diseases was raised, especially XN due to its association with MM. When performing a biopsy of the lesion, fragmentation of dermal collagen bundles and around vessels a pale eosinophilic acellular material, positive congo red and apple-green color with polarized light are observed in the histology, reaching the diagnosis of Amyloidosis and Orbital Xantogranuloma of the Adult.

Necrobiotic Xanthogranuloma Coexists with Diffuse Normolipidemic Plane Xanthoma and Multiple Myeloma

Necrobiotic xanthogranuloma associated with smoldering multiple myeloma: satisfactory response to cyclophosphamide, dexamethasone, and thalidomide

Abstract: Necrobiotic xanthogranuloma is a rare chronic condition, belonging to the group C non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly hematologic malignancies. The case reported here was only diagnosed after nine years of evolution and was associated with plasma cell dyscrasia. After treatment with cyclophosphamide, dexamethasone, and thalidomide, there was a reduction of cutaneous lesions and serum levels of monoclonal protein.

Primary Necrobiotic Xanthogranulomatous Sialadenitis with Submandibular Gland Localization without Skin Involvement

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.

Histiocitosis de células no Langerhans / Non-Langerhan cell histiocytoses

Las histiocitosis son un conjunto de enfermedades caracterizadas por la proliferación de células del sistema mononuclear fagocítico. Dentro de éstas, las histiocitosis de células noLangerhans (HCNL) son un grupo heterogéneo de entidades distintas, infrecuentes, que se caracterizan fundamentalmente por su naturaleza reactiva. No presentan evidenciasclínicas o de laboratorio de malignidad y tampoco proliferación de células de Langerhans. Nos referiremos en particular a cuatro entidades de este grupo: xantogranuloma juvenil(XJ), histiocitoma eruptivo generalizado (HEG), xantogranuloma necrobiótico (XN) y enfermedad de Rosai Dorfman (ERD) variedad cutánea pura...

Necrobiotic Xanthogranuloma with Monoclonal Gammopathy / 대한피부과학회지

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.

A Case of Post-traumatic Necrobiotic Xanthogranuloma of the Orbit

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.

Necrobiotic Xanthogranuloma with Multiple Myeloma and No Periorbital Involvement

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.

A Case of Pedunculated Xanthogranuloma in the Upper Eyelid Margin of an Adult / 대한피부과학회지

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.

A Case of Necrobiosis Lipoidica with Prominent Cholesterol Cleft / 대한피부과학회지

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.