Xanthoma Disseminatum

Frank, Samuel B.

doi:10.1001/archderm.1952.01530200092013

Cited by 22 publications

(3 citation statements)

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“…The lesions are usually asymptomatic and often appear in the periorbital area. In a few cases, systemic involvement was found in autopsies [6][7][8]. The most common extracutaneous localizations comprise the oropharyngeal tract, the bronchi, liver, spleen, lung and heart [9][10][11][12][13] Histopathologically, NXG is characterized by granulomas in the dermis extending into the subcutaneous fat.…”

Section: Introductionmentioning

confidence: 99%

Systemic therapy of necrobiotic xanthogranuloma: a systematic review

Steinhelfer

Kühnel²,

Jägle

et al. 2022

Orphanet J Rare Dis

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Background Even though a plethora of systemic therapies have been proposed for necrobiotic xanthogranuloma (NXG), there is no systematic review on this topic in literature. Objective To review all existing literature on the systemic therapy of NXG in order to identify the most effective therapies. Methods All reported papers in the literature were screened for systemic treatments of NXG. Papers without proper description of the therapies, papers describing topical therapy, and articles without assessment of effectiveness were excluded. Subsequently, we analyzed 79 papers and a total of 175 cases. Results The most effective treatments for NXG are intravenous immunoglobulins (IVIG), corticosteroids, and combination therapies including corticosteroids. Conclusions Corticosteroids and IVIG should therefore be considered first-line treatments in patients with NXG.

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Section: Introductionmentioning

confidence: 99%

Systemic therapy of necrobiotic xanthogranuloma: a systematic review

Steinhelfer

Kühnel²,

Jägle

et al. 2022

Orphanet J Rare Dis

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“…[13][14][15][16][17][18][19][20] The myocardium, skeletal muscles, kidneys, stomach, gallbladder, ovaries, lymph nodes, pancreas, uterus and suprarenal glands are other affected organs observed in post-mortem studies. [21][22][23] The possible involvement of the spinal cord in XD has not been well established. 15 At least six cases of XD with a papular onset, resembling GEH or papular xanthoma, have been previously described.…”

Section: Discussionmentioning

confidence: 99%

Systemic xanthohistiocytoma: a variant of xanthoma disseminatum?

Ferrando

Campo‐Voegeli²,

Soler-Carrillo³

et al. 1998

Br J Dermatol

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We present a 66-year-old man who had maculopapular pigmented lesions on the skin of the head, neck and trunk suggesting generalized eruptive histiocytoma (GEH). These lesions had a yellowish centre in a target-like pattern that has not been previously described. The patient suffered from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs. The explorations performed disclosed the presence of specific xanthomatous infiltrates in the skin, lungs, respiratory tract, peripheral nerves and meninges, suggesting xanthoma disseminatum (XD) or juvenile xanthogranuloma. Multiple osteolytic lesions of large bones were also found. The infiltrate was CD68, MAC 387 and factor XIIIa positive and S-100 and CD1 negative. Some cells contained worm-like bodies visible by electron microscopy. Our patient presented clinical and immunohistochemical findings suggestive of GEH, juvenile xanthogranuloma or XD, supporting the idea of a wide spectrum of non-Langerhans cell histiocytosis. These specific target-like xanthomatous lesions seem to be unique for this new variant of XD.

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“…In 1960, Halprin and Lorincz documented the first case of disseminated xanthosiderohistiocytosis (DXSH), a rare variant of xanthoma disseminatum (XD) with a distinct clinical and histopathologic presentation. To our knowledge, only 5 cases have been reported in the literature, with the most recent reported case dating back more than 25 years . We describe a 65-year-old man with DXSH in association with monoclonal gammopathy and ocular manifestations.…”

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confidence: 93%