ABSTRACT
No abstract available.
Subject(s)
Histiocytosis , Histiocytosis, Non-Langerhans-Cell , Stomach , XanthomatosisABSTRACT
Objective To explore the clinicopathological features and imaging characteristics of non-Langerhans cell histiocytosis in central nerve system,thus to facilitate the diagnosis and differential diagnosis.Methods A total of ten cases were enrolled in the study,with seven cases of Rosai-Dorfman disease(RDD) and three cases of xanthoma disseminatum (XD).Data on the clinicopathological features,imaging,immunophenotype and prognosis were collected and analyzed.Results Seven patients with RDD,5 males and 2 females with the mean age of 46.7 years old,all presented as dural-based or intraparenchymal hypo-to isointense lesions on T1 and T2 with post-contrast enhancement.The polymorphous admixture of histiocytes,lymphocytes and plasma cells was observed in a fibrous stroma,with emperipolesis of some histiocytes.The immunohistostaining of CD11c,CD68,MAC387 and S-100 was positive in the histiocytes,while the staining of CD1α was negative.Five patients recovered after the operation,while one patient died of the disease.All the 3 XD patients were female,with the median age of 20.7 years old.All XD patients presented as multiple intraparenchymal hypointense lesions on T1 and hyperintense lesions on T2 with post-contrast enhancement.The infiltration of foam-like histiocytes,a few Touton giant cells,lymphocytes and eosnophils was observed in all XD patients.The immunohistostaining of CD68 and CD11c was positive in the histiocytes and that of MAC387 partly positive,while the staining of S-100 and CD1α was negative.One XD patient survived well,while another one died of the disease.Conclusions The diagnosis of RDD and XD should be based on their typical morphology and immunophenotype and should be differentiated from Langerhans cell histiocytosis and other non-Langerhans cell histiocytosis.Non-Langerhans cell histiocytosis in central nerve system often presents untypical clinical presentation and imaging features,thus the communication and cooperation between clinician and pathologist is needed.
ABSTRACT
Xanthoma disseminatum (XD) is a rare, benign non-familial mucocutaneous disorder, which is a subset of non-Langerhans cell histiocytosis. It is characterized by mucocutaneous xanthomas in a disseminated form typically involving the eyelids, trunk, face, and proximal extremities and occurs in flexures and folds such as axillae and the groin. Mucosal involvement of the respiratory or gastrointestinal tracts may lead to hoarseness or intestinal obstruction from a mechanical mass effect. This paper outlines the case of a 47-year-old female with progressive yellow-to-brown confluent nodules and plaques of various sizes on her scalp, face, oral mucosa, neck, shoulder, axillary folds, and perianal area. Xanthomas accentuating over the eyelids and eyelashes led to partial obstruction of her visual field and interfered with blinking. Further, she suffered from xerophthalmia. The presentation of histopathological features including foamy histiocytes, inflammatory cells, and Touton giant cells in conjunction with her clinical findings indicated a diagnosis of XD. Evaluations for extracutaneous involvement including the central nervous system, respiratory tract, gastrointestinal tract, and bone resulted in nonspecific findings. Although she has been treated with surgical excisions, CO2 laser therapy, and oral prednisolone, new lesions are still emerging.
Subject(s)
Female , Humans , Middle Aged , Axilla , Blinking , Central Nervous System , Extremities , Eyelashes , Eyelids , Gastrointestinal Tract , Giant Cells , Groin , Histiocytes , Histiocytosis , Histiocytosis, Non-Langerhans-Cell , Hoarseness , Intestinal Obstruction , Lasers, Gas , Mouth Mucosa , Neck , Prednisolone , Respiratory System , Scalp , Shoulder , Visual Fields , Xanthomatosis , XerophthalmiaABSTRACT
Xanthoma disseminatum (XD) is a rare, potentially progressive non-Langerhans cell histiocytosis, which preferentially affects males in their childhood or young adulthood. It is characterized by the insidious onset of small, yellowish-red to brown papules and nodules that are discrete and disseminated. They characteristically involve the eyelids and flexural areas of the axillary and inguinal folds, and the antecubital and popliteal fossae. Systemic disease frequently occurs, the most common manifestation being diabetes insipidus (DI), which occurs in about 40% of cases and is due to deposition of xanthoma cells in the hypothalamic-pituitary axis. We present a case of XD with DI, which was treated with the combination therapy of CO2 laser vaporization, cyclophosphamide, electrocauterization and synthetic anti-diuretic hormone.
Subject(s)
Humans , Male , Axis, Cervical Vertebra , Cyclophosphamide , Diabetes Insipidus , Eyelids , Histiocytosis , Histiocytosis, Non-Langerhans-Cell , Lasers, Gas , Volatilization , XanthomatosisABSTRACT
Xanthoma disseminatum is a rare mucocutaneous xanthomatosis classified as a benign form of non-Langerhans cell histiocytosis. We describe an illustrative case with extensive mucocutaneous involvement which was unresponsive to treatment. The patient presented with numerous variable-sized, yellow-brown papules and confluent plaques on the perorbital, perioral, neck, axilla, upper chest, groin, perianal, antecubital fossae and popliteal fossae including the upper respiratory tract. She was treated with oral cyclophosphamide but showed no clinical improvement. She then developed dyspnea, dysphagia and experience defecation difficulties, so was treated with palliative surgery to help relieve the symptoms.
Subject(s)
Humans , Axilla , Cyclophosphamide , Defecation , Deglutition Disorders , Dyspnea , Groin , Histiocytosis , Histiocytosis, Non-Langerhans-Cell , Neck , Palliative Care , Respiratory System , Thorax , XanthomatosisABSTRACT
Xanthoma disseminatum is a rare benign, normolipemic mucocutaneous xanthomatosis resulting due to the proliferation of non-Langerhans cell histiocytes. Xanthoma disseminatum typically involves the skin, particularly the flexor skin folds. Mucocutaneous involvement develops in 40-60% of patients, most commonly affecting the oropharynx, larynx, or corneae and conjunctivae. Xanthoma disseminatum may also manifest in the central nervous system. While the natural history of xanthoma disseminatum is usually benign, lesions in critical anatomical locations may result in morbidity and mortality. Curative therapy is lacking. We report a case with laryngeal involvement in a female patient. In this patient, xanthomas involved oropharynx and larynx mucosa. Dyspnea occurred due to epiglottis lesion. Tracheotomy was performed, and maintenance of breathing and decanulation were possible after epiglottectomy.
Subject(s)
Female , Humans , Central Nervous System , Conjunctiva , Cornea , Dyspnea , Epiglottis , Histiocytes , Histiocytosis, Non-Langerhans-Cell , Larynx , Mortality , Mucous Membrane , Natural History , Oropharynx , Respiration , Skin , Tracheotomy , XanthomatosisABSTRACT
Xanthoma disseminatum (XD) is one of cutaneous nonhistiocytosis X, and characterized by multiple, widely distributed red, brown, yellowish papules and nodules that tend to coalesce. We present a case of XD with characteristic clinical findings and treated with the combination therapy of COz laser vaporization and high dose steroid.
Subject(s)
Histiocytosis, Non-Langerhans-Cell , Laser Therapy , Lasers, Gas , Volatilization , XanthomatosisABSTRACT
Xanthoma disseminatum is a rare benign, normolipemic form of histiocytoxanthomatosis, affecting the skin and mucous membranes, frequently associated with diabetes insipidus. It occurs mainly in young to middle-aged adults. The cutaneous manifestations consist of hundreds of yellowish papules which symmetrically involve the trunk, face, and proximal extremities including flexures and folds. The histopathologic finding is a mixture of foamy cells, histiocytes, and inflammatory cells. We report a 6-year-old male who had disseminated asymptomatic yellowish papules and plaques on whole body including flexures since infancy. Histological examination showed infiltrating foamy cells with lymphocytes and histiocytes in upper dermis. He had normal lipid profiles and symptoms of polyuria or polydipsia were absent.
Subject(s)
Adult , Child , Humans , Male , Dermis , Diabetes Insipidus , Extremities , Histiocytes , Histiocytosis, Non-Langerhans-Cell , Lymphocytes , Mucous Membrane , Polydipsia , Polyuria , Skin , XanthomatosisABSTRACT
Xanthoma disseminatum is a rare disorder that is a distinct subset of cutaneous nonhistiocytosis X. We report a case of xanthoma disseminatum in a 52-year-old woman. She had yellow-brownish variable sized papules or confluent plaques with a well-defined border on her periorbital, perioral, axilla, oral mucosa, neck, scalp, and upper chest areas. There were mixed infiltrations of foamy histiocytes, inflammatory cells, and Touton giant cells in the biopsy specimen. She had been treated with oral fenofibrate but showed no definite effect.
Subject(s)
Female , Humans , Middle Aged , Axilla , Biopsy , Fenofibrate , Giant Cells , Histiocytes , Histiocytosis, Non-Langerhans-Cell , Mouth Mucosa , Neck , Scalp , Thorax , XanthomatosisABSTRACT
We report a case of xanthoma disseminatum in a 24 year old male paitient. Multiple yellow-brown papules developed on the flexor aurfaces, such as the neck, axillae, antecubital fossae, groin, and perianal regions. Some papules were detected arouns the eyes and uvulai. biopsy specimen revealed a dense infiltrate of histiocytes, foam cells, Touton giant cells, and other inflammatory cells. No Langerhans granules were seen in the electron microscopic analysis.