Reduced Skin Capillary Density During Attacks of Erythromelalgia Implies Arteriovenous Shunting as Pathogenetic Mechanism

Mørk, Cato; Kvernebo, Knut; Asker, Claes; Salerud, E. Göran

doi:10.1046/j.1523-1747.2002.00218.x

Cited by 45 publications

(61 citation statements)

References 17 publications

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“…In a prospective study, Kalgaard et al (15) reported histopathological findings characterized by capillary proliferation or vascular damage in 31 of 49 specimens, mainly from patients with PEM. These nonspecific alterations indicated the presence of skin hypoxia secondary to increased arteriovenous shunting and insufficient capillary flow, which is compatible with the shunting hypothesis supported by Mørk et al (13,14).…”

Section: Discussionsupporting

confidence: 86%

“…According to the shunting hypothesis, EM symptoms are caused by tissue hypoxia, which is induced by a maldistribution of skin microvascular blood flow with increased thermoregulatory flow through arteriovenous shunts and an inadequate nutritive perfusion to normal skin capillaries (13,14). If available blood is shunted away from normal skin capillaries, the skin will be hypoxic.…”

Section: Discussionmentioning

confidence: 99%

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Secondary erythromelalgia successfully treated with patient-controlled epidural analgesia and interferon α-2b: A case report and review of the literature

Li²,

Yao

et al. 2016

Experimental and Therapeutic Medicine

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Abstract. Erythromelalgia (EM) is a debilitating neurovascular disease that is refractory to numerous treatment modalities. The present study reported the case of a 72-year-old woman diagnosed with EM secondary to polycythemia vera (PV), who was effectively treated with the use of patient-controlled epidural analgesia (PCEA) and interferon α-2b therapy. The study aimed to provide clinical information on this condition based on the findings of the present case and previously-reported cases. PCEA containing local anesthetics and dexamethasone or fentanyl resulted in nearly complete relief of symptoms. Furthermore, continuous use of interferon α-2b for the treatment of the PV completely relieved the EM symptoms. In conclusion, PCEA and interferon α-2b may be promising treatments for EM secondary to PV.

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Secondary erythromelalgia successfully treated with patient-controlled epidural analgesia and interferon α-2b: A case report and review of the literature

Li²,

Yao

et al. 2016

Experimental and Therapeutic Medicine

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“…15 While the alteration of the sympathetic neurons' function leads to the microvascular symptoms, the alteration of the nociceptive neurons' function results in severe burning pain, characterizing erythromelalgia. 10 Despite an increase blood flow (as measured with laser Doppler), there is local hypoxia that can be reflected by low TCPO 2 values, which might be explained by 29 Unlike the primary type, secondary type pathophysiology is poorly understood and thought to be due to neuropathological and microvascular functional changes caused by the underlying condition. 30 Since there is no diagnostic test for erythromelalgia, a very good history and physical exam are crucial for the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Severe case and literature review of primary erythromelalgia: Novel SCN9A gene mutation

et al. 2011

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A 15-year-old female presented with a 13-year history of chronic intermittent painful skin redness and swelling of both feet and lower legs.Her problem began when she started to walk. She had difficulty with, and avoided walking on rough textures and warm surfaces. She also cried when rough textured clothing was placed on the lower legs and feet. At the age of 4, the patient started to have frequent episodes of pain in the lower legs and feet with associated erythema, edema and warmth. Symptoms were present more often during the day and were aggravated by warm temperatures. She found symptomatic relief by immersing her feet in cold water. There were no other reported skin abnormalities or complaints, including joint pains, or constitutional symptoms including weight loss, fever, chills or fatigue. Her family history was negative for similar symptoms or vascular disorders.For the subsequent 9 years, the patient had continued to have similar symptoms intermittently that did not affect her daily activities, school performance, or function. She was later diagnosed with hypothyroidism that was easily controlled with thyroid hormone supplement. Her exam was otherwise unremarkable with the exception of her small stature, placed on the fourth and 37th percentiles respectively for height and weight. The patient was diagnosed with growth hormone deficiency. IGF-1 values were low at 218 ng/ml (range: 228-957). She underwent growth hormone stimulation testing with clonidine, which showed her peak growth hormone response was 0.5 ng/ml (normal peak is greater than 10). A sustained trial of growth hormone replacement was not done at the family's preference.At the age of 13 years, the patient started to have intermittent blood pressure elevations that were worse at the times of her symptoms (highest systolic around 180). She used to receive intravenous nitroprusside that seemed to alleviate her skin symptoms. Her secondary hypertension work-up revealed normal electrolyte panels, urinalysis, renal ultrasound, and cardiac echocardiograms. However, during painful crises, plasma free total metanephrine (350 pg/ml; ref. range: < 206) and plasma free normetanephrine (324 pg/ml; ref. range: < 149) were noted to be increased. Abstract Erythromelalgia is a rare clinical syndrome characterized by intermittent heat, redness, swelling and pain more commonly affecting the lower extremities. Symptoms are mostly aggravated by warmth and are eased by a cold temperature. In some cases, symptoms can be very severe and disabling. Erythromelalgia can be classified as either familial or sporadic, with the familial form inherited in an autosomal dominant manner. Recently, there has been a lot of progress in studying Na(v)1.7 sodium channels (expressed mostly in the sympathetic and nociceptive small-diameter sensory neurons of the dorsal root ganglion) and different mutations affecting the encoding SCN9A gene that leads to channelopathies responsible for some disorders, including primary erythromelalgia. We present a severe case of progressive primar...

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“…The skin, and the dermis in particular, is a very active part of both the antigen-specific and innate immune system that can interfere with the metastatic process. On the other hand, the skin is one of the most capillarized tissues in humans with a density of 70-90 capillaries per mm 2 [31,32]. Considering the spatial limits of oxygen and nutrient diffusion (around 100 μm), disseminated cancer cells in the skin do not depend on their own angiogenic potential.…”

Section: Blood Perfusion Ratementioning

confidence: 99%

“…Reports on the comparison of early and late stage disease CTC numbers found limited correlation [36,37]. With advances of cell isolation technologies, the detection threshold has become lower, reaching 1-5 CTC/10 ml blood [32]. It is a well-established fact that the metastatic efficiency of hematogenous dissemination is 0.01 %; in other words, 1 cell out of 10,000 tumor cells in the circulation [6,7], indicating that the overwhelming majority of CTC is incapable of colonizing the distant organs.…”

Section: Circulating Cancer Cellsmentioning

confidence: 99%

Tumor type-specific and skin region-selective metastasis of human cancers: another example of the “seed and soil” hypothesis

Kovács

Hegedűs

Kenessey

et al. 2013

Cancer Metastasis Rev

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Metastasis of human cancer is an organ-selective process that is determined by anatomical and biological factors as well as by specific microenvironmental properties. Dissemination of visceral malignancies to the skin is rather rare and usually occurs in a later stage of the disease. Using statistical approaches, both positive (renal and lung cancers) and negative (pancreatic and liver cancers) organ preferences can be identified in a variety of cancers. While certain cancer types are characterized by random distribution for skin metastasis (liver cancer), a number of cancers demonstrate a colonization preference to the region of origin: lung cancer to the supradiaphragmatic (mostly chest) and colorectal cancers to the infradiaphragmatic (abdominal) skin regions. In certain cases, however, skin metastasis develops more frequently at specific distant locations, as evidenced by the dissemination of renal cancer at the head and neck region. These findings are clinically relevant and useful especially in patients where skin metastasis is the first indication of a malignancy. Nevertheless, it is a strong argument for the predominant role of microenvironmental factors in cancer dissemination. On the other hand, skin metastases of visceral cancers provide a unique model to analyze the pathomechanisms determining organ selectivity, including the organ-specific vascularization, the dermatome-specific innervation, or immunological and developmental factors.

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Reduced Skin Capillary Density During Attacks of Erythromelalgia Implies Arteriovenous Shunting as Pathogenetic Mechanism

Cited by 45 publications

References 17 publications

Secondary erythromelalgia successfully treated with patient-controlled epidural analgesia and interferon α-2b: A case report and review of the literature

Secondary erythromelalgia successfully treated with patient-controlled epidural analgesia and interferon α-2b: A case report and review of the literature

Severe case and literature review of primary erythromelalgia: Novel SCN9A gene mutation

Tumor type-specific and skin region-selective metastasis of human cancers: another example of the “seed and soil” hypothesis

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