Lymphatic microangiopathy of the skin in systemic sclerosis

Leu, Anders J.; Gretener, Silvia B.; Enderlin, S; Brühlmann, P; Michel, Beno; Kowal-Bielecka, Otylia; Hoffmann, Ulrich; Uk, Franzeck

doi:10.1093/rheumatology/38.3.221

Cited by 22 publications

(20 citation statements)

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“…While the role of blood vessel and vessel dysfunction in the pathogenesis of SSc has been the object of much investigation, only a microlymphography study by Leu et al [22] focuses on lymphatic vessels in this disease. The present immunohistochemical study is, to the best of our knowledge, the first detailed expression and morphometric analysis of lymphatic vessels in the skin of patients affected by SSc.…”

Section: Discussionmentioning

confidence: 99%

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Lymphatic and blood vessels in scleroderma skin, a morphometric analysis

Rossi¹,

Sozio²,

Sestini³

et al. 2010

Human Pathology

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SummaryVascular involvement is frequent in systemic sclerosis, but the role of the lymphatic vasculature is poorly known. Our aim was to evaluate lymphatic vessels in systemic sclerosis skin lesions. We studied skin forearm biopsies of 9 patients with systemic sclerosis and 7 age-matched controls. Lymphatic vessels were labeled with the monoclonal antibody D2-40 and blood vessels with a polyclonal antibody to von Willebrand Factor. All blood and lymphatic vessels present in each section were counted and total area, inner luminal area, and shape factors were measured. The number of blood and lymphatic vessels in papillary dermis was greater and their diameter lower than in reticular dermis both in systemic sclerosis and controls. In the reticular dermis, the number of lymphatic vessels was markedly reduced in systemic sclerosis (4.9 ± 1.1 μm−2 versus 8.9 ± 1.2 μm−2P = .03), and a similar trend was observed in papillary dermis (8.4 ± 3.7 μm−2 versus 8.1 ± 5.3 μm−2). Interestingly, the number of periglandular lymphatics in systemic sclerosis was not different from controls. The inner luminal area (possibly indicating compensatory dilation) of lymphatic vessels, particularly the periglandular ones, was greater in systemic sclerosis than in controls. No differences were observed in the number of blood vessels, but the percentage of blood vessel profiles (without lumen) was significantly less in systemic sclerosis both in papillary and in reticular dermis. In conclusion, our data show that skin lesions in systemic sclerosis are characterized by a selective rarefaction of lymphatic vasculature that spares periglandular vessels and that might have a pathogenic role in the evolution and in the clinical manifestations of the disease.

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Section: Discussionmentioning

confidence: 99%

“…To the best of our knowledge, the only report on lymphatic vessels in the skin of SSc patients is a fluorescence microlymphography study by Leu et al [22]. A solution of fluorescein isothiocyanate-dextran was injected into the subepidermal layer of the skin of fingers, hands and forearms.…”

Section: Introductionmentioning

confidence: 99%

Lymphatic and blood vessels in scleroderma skin, a morphometric analysis

Rossi¹,

Sozio²,

Sestini³

et al. 2010

Human Pathology

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“…Consistent with our results obtained by immunostaining, a previous case report described rarefication of lymphatic vessels in a patient with SSc, as assessed by conventional lymphangiography (25). Moreover, impaired cutaneous transport of fluorescein isothiocyanate–labeled dextran has been described in patients with SSc (26). The severity of the lymphangiopathy in SSc is underlined by the fact that the relative reduction in the number of lymphatic vessels was more pronounced than the reduction in the blood vessel count, with a decrease of 70% in lymphatic vessels as compared with a 25% reduction in blood vessels.…”

Section: Discussionmentioning

confidence: 99%

Decreased lymphatic vessel counts in patients with systemic sclerosis: Association with fingertip ulcers

Akhmetshina

Beer

Zwerina

et al. 2010

Arthritis & Rheumatism

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Objective. Systemic sclerosis (SSc) is a connective tissue disease that is characterized by microvascular disease and tissue fibrosis. Progressive loss and irregular architecture of the small blood vessels are well characterized, but the potential involvement of the lymphatic vessel system has not been analyzed directly in SSc. This study was undertaken to assess whether the lymphatic vascular system is affected in SSc, and whether changes to the lymphatic vessels are associated with dystrophic changes and tissue damage in patients with SSc.Methods. Lymphatic endothelial cells in skin biopsy samples from patients with SSc and age-and sex-matched healthy volunteers were identified by staining for podoplanin and prox-1, both of which are specifically expressed in lymphatic endothelial cells but not in blood vascular endothelial cells. CD31 was used as a pan-endothelial cell marker. Statistical analyses were performed using Kruskal-Wallis, Mann-Whitney U, and Spearman's rank correlation tests.Results. The numbers of podoplanin-and prox-1-positive lymphatic vessels were significantly reduced in patients with SSc as compared with healthy individuals. The number of podoplanin-positive lymphatic precollector vessels was significantly lower in SSc patients with fingertip ulcers than in SSc patients without ulcers. Moreover, the number of lymphatic vessels correlated inversely with the number of fingertip ulcers at the time of biopsy and with the number of fingertip ulcers per year. The inverse correlation between lymphatic precollector vessel counts and fingertip ulcers remained significant after statistical adjustment for the blood vessel count, age, and modified Rodnan skin thickness score.Conclusion. These results demonstrate a severe reduction in the number of lymphatic capillaries and lymphatic precollector vessels in patients with SSc.

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“…Microlymphography (11) showed that vessel alterations were related to disease progression, and histopathologic studies demonstrated a rarefaction of skin lymphatic vessels (12,13) and an inverse correlation of the number of lymphatic vessels with the number of ulcers per year (13).…”

Section: Introductionmentioning

confidence: 99%

“…Since the intricate pathogenic pathway of edema in SSc may comprehend microvascular changes (both at blood [14] and lymphatic [11][12][13] vessels), increased sympathetic activity (15), and inflammatory changes (16), we hypothesized that finger and hand edema present in SSc patients might respond to MLD, potentially acting on these aspects.…”

Section: Introductionmentioning

confidence: 99%

Manual lymph drainage improving upper extremity edema and hand function in patients with systemic sclerosis in edematous phase

Bongi¹,

Rosso²,

Passalacqua³

et al. 2011

Arthritis Care & Research

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Objective. In systemic sclerosis (SSc; scleroderma) patients in edematous phase, hand edema is often present. Manual lymph drainage (MLD) stimulates the lymphatic system and reduces edema. Our aim was to evaluate the efficacy of MLD in reducing edema and in improving functionality of the hands and perceived quality of life (QOL) in SSc patients in edematous phase. Results. In the intervention group, hand volume, the HAMIS test, and the 4 VAS were improved significantly at the end of treatment (P < 0.001). The results were maintained at T2 (P < 0.001). The HAQ and the PSI and MSI of the SF-36 also improved significantly at T1 (P < 0.001), but only PSI improvement was maintained at T2 (P < 0.001). In the observation group, no improvement at T1 and at T2 was observed. Conclusion. In SSc, MLD significantly reduces hand edema and improves hand function and perceived QOL.

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Lymphatic microangiopathy of the skin in systemic sclerosis

Abstract: Lesional skin in patients with systemic sclerosis exhibits evidence of lymphatic microangiopathy.

Cited by 22 publications

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Lymphatic and blood vessels in scleroderma skin, a morphometric analysis

Lymphatic and blood vessels in scleroderma skin, a morphometric analysis

Decreased lymphatic vessel counts in patients with systemic sclerosis: Association with fingertip ulcers

Manual lymph drainage improving upper extremity edema and hand function in patients with systemic sclerosis in edematous phase

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