Treatment of Mycosis Fungoides with Bexarotene Results in Remission of Diffuse Plane Xanthomas

Gregoriou, Stamatis; Rigopoulos, Dimitrios; Stamou, Christos; Nikolaou, Vasiliki; Kontochristopoulos, George

doi:10.2310/7750.2012.12022

Cited by 8 publications

(5 citation statements)

References 6 publications

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“…Cladribine has been used successfully in Langerhans cell histiocytosis of different types including plane xanthoma [ 10 ]. There are case reports on regression of DNPX during treatment (of associated disorders) with cyclosporine A [ 11 ] or bexarotene [ 12 ]. DNPX can be treated by ablative lasers such as erbium-YAG laser [ 13 ][ 14 ].…”

Section: Discussionmentioning

confidence: 99%

Diffuse Normolipemic Plane Xanthoma (DNPX) of the Neck without Xanthelasma Palpebrum

Wollina¹,

Schönlebe²,

Tchernev³

et al. 2018

Open Access Maced J Med Sci

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Diffuse normolipemic plane xanthoma (DNPX) is an uncommon subtype of non-Langerhans histiocytosis. DNPX is characterised by xanthelasma palpebrarum, diffuse plane xanthoma of the head, neck, trunk, or extremities, and normal plasma lipid levels. The neck is the most common site. We report about a 62-year-old female Caucasian patient, who developed an asymptomatic fine wrinkling and loose skin on the neck and décolleté about three years ago. The skin colour became yellowish. Xanthelasma was absent. Histopathology of a skin biopsy confirmed the diagnosis of DNPX. The patient had a medical history of chronic myeloblastic leukaemia. No other laboratory abnormalities were found. Laser treatment was offered but opposed by the patient.

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Section: Discussionmentioning

confidence: 99%

Diffuse Normolipemic Plane Xanthoma (DNPX) of the Neck without Xanthelasma Palpebrum

Wollina¹,

Schönlebe²,

Tchernev³

et al. 2018

Open Access Maced J Med Sci

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“…Both are nuclear receptors which regulate the transcription of apoE and ABCA1 (Liang et al, 2004; Zhao et al, 2014) by forming heterodimers with each other, or with other nuclear receptors such as retinoid acid receptor (RAR), peroxisome proliferator-activated receptor γ (PPARγ), thyroid hormone receptor (TR) or vitamin D receptor (VDR) as transcriptional co-regulators (Chawla et al, 2001; Evans and Mangelsdorf, 2014; Sussman and de Lera, 2005; Szanto et al, 2004). Of importance, bexarotene, an RXR agonist, has been reported to improve cognitive function as well as reduce brain amyloid-β (Aβ) levels in an Alzheimer’s disease (AD) mouse model (Cramer et al, 2012), although it was originally approved by the U.S. Food and Drug Administration (FDA) to treat cutaneous T-cell lymphoma (Gregoriou et al, 2013; Mehta et al, 2012; Scarisbrick et al, 2013; Vakeva et al, 2012). However, the effects of bexarotene on AD are controversial due to conflicting results from different investigators (Fitz et al, 2013; LaClair et al, 2013; Price et al, 2013; Tesseur et al, 2013; Veeraraghavalu et al, 2013).…”

Section: Introductionmentioning

confidence: 99%

Rescuing effects of RXR agonist bexarotene on aging-related synapse loss depend on neuronal LRP1

Tachibana

Shinohara

Yamazaki

et al. 2016

Experimental Neurology

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Apolipoprotein E (apoE) plays a critical role in maintaining synaptic integrity by transporting cholesterol to neurons through the low-density lipoprotein receptor related protein-1 (LRP1). Bexarotene, a retinoid X receptor (RXR) agonist, has been reported to have potential beneficial effects on cognition by increasing brain apoE levels and lipidation. To investigate the effects of bexarotene on aging-related synapse loss and the contribution of neuronal LRP1 to the pathway, forebrain neuron-specific LRP1 knockout (nLrp1−/−) and littermate control mice were administered with bexarotene-formulated diet (100 mg/kg/day) or control diet at the age of 20-24 months for 8 weeks. Upon bexarotene treatment, levels of brain apoE and ATP-binding cassette sub-family A member 1 (ABCA1) were significantly increased in both mice. While levels of PSD95, glutamate receptor 1 (GluR1), and N-methyl-D-aspartate receptor NR1 subunit (NR1), which are key postsynaptic proteins that regulate synaptic plasticity, were decreased with aging, they were restored by bexarotene treatment in the brains of control but not nLrp1−/− mice. These results indicate that the beneficial effects of bexarotene on synaptic integrity depend on the presence of neuronal LRP1. However, we also found that bexarotene treatment led to the activation of glial cells, weight loss and hepatomegaly, which are likely due to hepatic failure. Taken together, our results demonstrate that apoE-targeted treatment through the RXR pathway has a potential beneficial effect on synapses during aging; however, the therapeutic application of bexarotene requires extreme caution due to its toxic side effects.

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“…Physical approaches and surgical resection are too invasive for general lesions. 6 Gregoriou et al 7 reported that bexarotene was effective for treating DPX. Oral probucol has also been proposed.…”

Section: Discussionmentioning

confidence: 99%

Simvastatin ointment in the treatment of seven childhood diffuse plane xanthomas

Xia

Yang

et al. 2020

The Journal of Dermatology

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Diffuse plane xanthomas (DPX) is a rare, chronic, metabolic skin disease which can seriously affect the appearance of the patients and result in psychological problems. Existing treatments are seriously limited. Our objective was to assess the efficacy of and tolerance to simvastatin ointment for the treatment of skin lesions in seven DPX patients. In this study, patients were treated twice daily on the right side of the lesions for 10 months with the 1% or 5% simvastatin ointment. Meanwhile, the left body was untreated as a contralateral controlled side. Efficacy was assessed via the change of rash and was further confirmed by histological study. For all seven participants, both the 1% and 5% simvastatin ointments were effective. The histological change in DPX showed significant decrease of foam cells in skin lesions. The immunohistochemical staining of CD68, macrophage scavenger receptor type 1, oxidized low‐density lipoprotein receptor 1 and Oil Red O became obviously reduced after treatment. The test medicines were safe with only some skin‐related side‐effects. This is the first research reported on simvastatin ointment treatment in DPX. Our results are the first to suggest that simvastatin ointment is effective in improving skin lesions of DPX clinically and histopathologically.

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Treatment of Mycosis Fungoides with Bexarotene Results in Remission of Diffuse Plane Xanthomas

Abstract: Bexarotene led to the clearing of the cutaneous lesions of cutaneous T-cell lymphoma and plane xanthomas. This may be due to an effect of bexarotene on the aberrant T cells that may cause xanthomatization.

Cited by 8 publications

References 6 publications

Diffuse Normolipemic Plane Xanthoma (DNPX) of the Neck without Xanthelasma Palpebrum

Diffuse Normolipemic Plane Xanthoma (DNPX) of the Neck without Xanthelasma Palpebrum

Rescuing effects of RXR agonist bexarotene on aging-related synapse loss depend on neuronal LRP1

Simvastatin ointment in the treatment of seven childhood diffuse plane xanthomas

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