Xanthoma Disseminatum

Altman, Jules

doi:10.1001/archderm.1962.01590110018003

Cited by 98 publications

(73 citation statements)

References 2 publications

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“…They appear concurrently with, or after, the cutaneous lesions. They have been re ported in 40-60% of cases [5,25] and in 4 of our 7 cases. The most frequently affected mucosal areas are, in order, the larynx, the pharynx, the mouth, the trachea, the con junctiva and cornea, the epiglottis and the tongue [25].…”

Section: ]supporting

confidence: 51%

“…From a literature review [5], it arises that approximately 60% of XD cases had their onset between the 5th and the 25th year of age. In our 5 adult cases and in most of those subsequently reported in the literature [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20], the average age of onset of the disease was over 40 years.…”

Section: Resultsmentioning

confidence: 99%

“…The most frequently affected mucosal areas are, in order, the larynx, the pharynx, the mouth, the trachea, the con junctiva and cornea, the epiglottis and the tongue [25]. Ocular lesions may cause obstructive blindness [26], while massive involvement of the upper respiratory tract may require tracheostomy (7 cases described, our patient in cluded) [ 17], Diabetes insipidus, reported in approximately 40% of patients [5,25], was present in 3 of our 7 cases; it is usually mild, transient and well controlled by vasopressin. It may be the first symptom of the disease and may precede the cutaneous lesions (like in our 3rd case).…”

Section: ]mentioning

confidence: 95%

“…According to the data reported by Altman and Winkelman [5], men outnumber women by a ratio of approxi mately 2 to 1 [2,6], XD is a mucocutaneous histiocytoxanthomatosis with an unmistakable clinical picture, due to the type, distribution and location of the lesions. The typical cutaneous orangeyellow-brownish papulonodules, initially isolated, gradu ally tend to coalesce and merge into plaques, mostly on the main folds, the process lasting from a few months to sev eral years.…”

Section: ]mentioning

confidence: 99%

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The Various Clinical Patterns of Xanthoma disseminatum

et al. 1995

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Background: Xanthoma disseminatum (XD) is a rare mucocutaneous xanthoma nowadays classified as a benign non-Langerhans cell histiocytosis. Objective: We studied 7 cases of XD with a long follow-up and reviewed the literature in order to compare and discuss previous reports. Methods: We studied clinical manifestations, course, histopathologic, ultrastructural and immunohistochemical findings of 7 cases of XD followed for 1–20 years. Results: We report our findings and compare our cases with those described in the literature. Conclusions: XD and Langerhans cell histiocytosis were distinguished by clinical differences, as well as ultrastructural and immunohistochemical methods. Three clinical patterns of XD appeared to emerge in our group of patients and in the cases reviewed from the literature.

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Section: ]supporting

confidence: 51%

Section: Resultsmentioning

confidence: 99%

Section: ]mentioning

confidence: 95%

Section: ]mentioning

confidence: 99%

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The Various Clinical Patterns of Xanthoma disseminatum

et al. 1995

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“…Contudo, o xantoma disseminado manifesta-se, freqüentemente, por numerosas pápulas coalescentes em áreas de flexão cutânea (11,12). Dois casos anteriormente descritos na literatura como exemplos da associação do xantoma disseminado ao DIC foram posteriormente questionados, pois, segundo a opinião de alguns autores, poderiam se tratar do xantoma papular, uma vez que não havia o infiltrado inflamatório e o quadro clínico característico do xantoma disseminado (2).…”

Section: Discussionunclassified

Histiocitose cutânea não-Langerhans como causa de diabetes insípido central

Miranda

Bittencourt

et al. 2007

Arq Bras Endocrinol Metab

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RESUMOAs histiocitoses são doenças raras, resultantes de alterações na linhagem monocítica-histiocítica, com manifestações clínicas diversas. Entre as síndromes cutâneas de células não-Langerhans, o xantoma disseminado é a única entidade desse grupo classicamente associada ao diabetes insípido central (DIC). O caso clínico relatado refere-se a um paciente de 30 anos de idade que, dois anos após o diagnóstico de DIC, evoluiu com lesões cutâneas papulosas, eritêmato-acastanhadas, difusas, discretas e não confluentes. Os achados histológicos, imuno-histoquímicos e a microscopia eletrônica mostraram resultados compatíveis com a histiocitose de células não-Langerhans e sugestivos do xantogranuloma juvenil. A avaliação endócrino-metabólica não mostrou alterações durante o seguimento por 10 anos, com exceção do DIC. A ressonância magnética da hipófise demonstrou ausência do sinal hiperintenso (mancha brilhante) correspondente à neuro-hipófise. As radiografias e a cintilografia dos ossos não mostraram lesões osteolíticas. Este caso desperta a atenção para a importância do exame da pele nos casos de DIC e de sua associação com a histiocitose de células não-Langerhans de maneira mais ampla, e não restrita aos casos de xantoma disseminado. ABSTRACT Cutaneous Non-Langerhans Cells Histiocytoses as Cause of Central Diabetes Inspidus.The histiocytoses are rare diseases caused by alterations in the monocytehistiocytic series with several clinical findings. Among the cutaneous syndromes of non-Langerhans cells, xanthoma disseminatum is the only disease of this group that has been classically associated to the central diabetes insipidus (CDI). The case reported describes a 30-year-old man that two years after presenting with CDI developed non confluent disseminated cutaneous brown papular lesions throughout the body. The histopathology, immunohistochemistry, and electronic microscopy were compatible with the diagnosis of non-Langerhans histiocytoses, suggesting the diagnosis of juvenile xanthogranuloma. The endocrine-metabolic evaluation did not show other alterations besides CDI in a 10-year follow up. The magnetic resonance of hypophysis showed absence of the pituitary hyperintense sign (bright spot). The radiologic and scinthigraphic evaluation of the bones did not show the presence of osteolytic lesions. This case prints out the importance of skin examination in cases of CDI and its association with cutaneous non-Langerhans histiocytoses in a broader spectrum, rather then restricted to the cases of xanthoma disseminatum.

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Cutaneous Syndromes of Non-X Histiocytosis

Winkelmann¹

1981

Arch Dermatol

153

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Xanthoma Disseminatum

Cited by 98 publications

References 2 publications

The Various Clinical Patterns of Xanthoma disseminatum

The Various Clinical Patterns of Xanthoma disseminatum

Histiocitose cutânea não-Langerhans como causa de diabetes insípido central

Cutaneous Syndromes of Non-X Histiocytosis

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