Lymphedema secondary to limited cutaneous systemic sclerosis

Abstract

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterised by vascular abnormalities, immune system activation and fibrosis. Lymphatic involvement in SSc was described more recently and starts in early stages. This report describes a 46-year-old patient who developed over the last 2 years asymmetric lymphedema in lower extremities. Compromise in lymphatic drainage was confirmed by lymphoscintigraphy. She also presented Raynaud's phenomenon, a scleroderma pattern in nailfold capillaroscopy, cutaneous thickening and anticentromere antibodies, which together resulted in a new diagnosis of limited cutaneous SSc. Treatment with methotrexate, prednisolone and lymphatic drainage resulted in lymphedema improvement. To our knowledge, this is the first case of grade 2 lymphedema in the setting of anticentromere-positive limited cutaneous SSc. We highlight the importance of considering rheumatic diseases in the differential diagnosis of lymphedema.

Lymphaticovenular anastomosis and venous arterialization in coexisting Raynaud's phenomenon and lymphedema: A case report

“…There have been only four case reports of comorbid Raynaud's phenomenon and lymphedema, all of which involved patients with other systemic disease, namely, yellow nail syndrome (Awerbuch, ), Fabry disease (Usmani, Camacho, Elfenbein, & Reginato, ), systemic sclerosis (Moreira, Crespo, & Santos, ), and metastasis of a malignant tumor (Al‐Nahhas, Collins, Jawad, & McCready, ). The only treatment options for these patients were medication for the systemic disease or palliative therapy.…”

Effect of Goreisan, a Traditional Japanese Medicine, on Rat Hindlimb Lymphedema

“…Although no appropriate medications for lymphedema exist, a few case studies reported that the diuretic FRS and the common anti-inflammatory corticosteroid PDN improved lymphedema. 37,38) In this study, we observed that FRS and PDN inhibited the increase in foot volume in hindlimb lymphedema rats. In support of the important role of inflammation, ketoprofen, a nonsteroidal anti-inflammatory drug (NSAID), restored edema and fibrosis in experimental lymphedema in mice due to the inhibition of 5-lipoxygenase-dependent leukotriene B4 (LTB4) production.…”