Lymphedema and Kaposi sarcoma: A narrative review.

BACKGROUND: Kaposi sarcoma (KS), due to HHV-8 infection is classified in 4 subtypes: epidemic, endemic, HIV-related and iatrogenic essentially after organ transplant. Lymphedema is a complication of KS. We reviewed the interactions between HHV-8 infection and lymphedema according an analysis of the literature. MAIN BODY: HHV-8 can infect different types of cells, among them a privileged tropism for lymphatic endothelial cells. It induces multi-centric endothelial proliferation leading to the occlusion of lymphatic vascular lumen. Lymphatic obstruction progressively lead to the blockage of lymphatic drainage, lymph stasis and lymphedema. Lymphedema mostly involved the lower limb affected by KS. It can then develop simultaneously or after the appearance of KS lesions but also be the first sign of KS, a long time before KS skin lesion onset. Lymphedema diagnosis is clinical and lymphoscintigraphy can confirm it if necessary. Lymphedema may be associated with active lesions of KS or non-evolutive, with only cicatricial lesions. KS should be treated according to the KS subtype, aggressive form, with local or systemic treatments associating with causal treatment, such as HIV infection or reducing immuno-suppressive drugs in transplant patients. In most of the cases, KS treatment may slightly reduce (or not) lymphedema volume which remains a chronic disease. Lymphedema management should be associated in order to reduce the volume and then stabilizing it. Low-stretch bandage, elastic garments and skin care are the cornerstone of treatment. CONCLUSION: Lymphedema is a frequent complication of KS, and may reveal KS or occurs throughout its course. Association of KS and lymphedema must be known because lymphedema is a chronic disease affecting the quality of life. Beyond the treatment of KS, its management must be specific including a long follow-up to optimize the patient's observance required to maintain the best lymphedema control.

[Pretibial myxedema after Graves' disease: A differential diagnosis of lymphedema]. / Myxœdème prétibial pseudo-tumoral après une maladie de Basedow : un diagnostic différentiel du lymphœdème.

INTRODUCTION: Pretibial myxedema is a rare manifestation of Graves' disease, and pseudotumoral forms may be confused with lower limb lymphedema. OBSERVATIONS: We reported 3 cases of pretibial myxedema in 2 women and 1 man, aged 72, 66, and 49 years, treated for Graves' disease 3, 25 and 32 years previously. Two patients were active smokers. Lymphedema diagnosis of the lower limbs was suspected in the presence of bilateral pseudotumoral lesions of the feet, toes and ankles and the presence of a Stemmer's sign (skin thickening at the base of the 2nd toe, pathognomonic of lymphedema). Lymphoscintigraphy in one case was normal, not confirming lymphedema. CONCLUSION: Pretibial pseudotumoral myxedema is a differential diagnosis of lower limb lymphedema. This diagnosis is confirmed by questioning the patient about preexisting Graves' disease, the underlying etiology, to decide the appropriate treatment and to encourage cessation of smoking, which is a risk factor for pretibial myxedema.

[Blue toe syndrome as an initial manifestation of metastatic prostate cancer : A case report with literature review]. / Un syndrome des orteils bleus révélant un cancer de la prostate métastatique : une observation avec analyse de la littérature.

INTRODUCTION: Cutaneous and vascular manifestations of cancer are numerous. Among paraneoplastic acral vascular syndrome, we report a case of blue toe syndrome as the first manifestation of a prostate cancer following with analysis of this syndrome according literature. OBSERVATION: A 56-year-old man, with Raynaud's phenomenon of the upper limbs for 2 to 3 years, had 4 blue toes of the left foot evolving for 18 months, without ulceration, the pulses being present. Vascular and cardiac explorations (ultrasound, angio-MRI) were normal. There was no biological or immunological abnormality except an elevated PSA level. Prostate biopsies confirmed diagnosis and abdomino-pelvic CT scan proved the bone and lymph node metastasis. CONCLUSION: The revelation of a prostate cancer with bone metastases by a blue toe syndrome is a rare situation. In a patient with a blue toe syndrome with no obvious clinical or biological abnormality, especially atheromatous, investigations should include a search for cancer, which can be revealed by blue toes.

[Puffy hand syndrome: A little-known diagnosis]. / Le syndrome des grosses mains : un diagnostic méconnu.

INTRODUCTION: Puffy hand syndrome is a rare complication of intravenous drug addiction. Diagnosis is based on the patient's history and clinical examination. OBSERVATIONS: A woman and two men, aged 42, 39 and 36 years old, are described. All had a history of intravenous drug use of heroin and oral buprenorphine misuse. Puffy hand syndrome appeared during drug addiction (n = 2) or after its withdrawal (n = 1). It was associated with acrocyanosis (n = 1) or injection scars (n = 1). Upper limb ultrasonography showed sequelae of venous (n = 3) or arterial (n = 1) thrombosis. An upper limb lymphoscintigraphy in one patient showed decreased radionuclide uptake of axillary lymph node and subdermal reflux tracer in the forearm. Treatment was based on low-stretch bandages to reduce the volume and then elastic compression sleeve for long-term stabilization. CONCLUSION: Puffy hand syndrome seen in intravenous drug addicts is poorly understood. It is a chronic complication despite the cessation of drug use. This syndrome has to become more widely known because its management is mandatory, although symptomatic.

[Lower limb lymphedema: A rare manifestation of spondyloarthropathy]. / Lymphœdème des membres inférieurs : une manifestation rare des spondyloarthrites.

INTRODUCTION: Spondyloarthritis include articular (axial, peripheral or enthesitic) and extra-articular manifestations. We reported three cases of limb lymphedema associated with a spondyloarthritis. CASE REPORTS: We report on two men and one female aged of 75, 52 and 39 years, respectively. The rheumatic disease was an ankylosing spondylitis HLA B27 positive in two patients and a psoriatic arthritis in the remaining one. Lymphedema, always unilateral, confirmed by the Stemmer's sign, involved the whole lower limb (n=2) or only the foot and calf (n=1). Lymphedema occurred 3 months, 40 and 9 years after the onset of spondyloarthritis. Lower limb lymphoscintigraphy was abnormal in all cases: decrease (n=2) or absence (n=1) of inguinal lymph node uptake, and visualization of the popliteal lymph node related to deep lymphatic pathway (n=1). Treatment of the spondyloarthritis did not improve lymphedema. Both the low-stretch bandage and elastic compression treatment allowed its reduction and stabilization. CONCLUSION: Limb lymphedema is a rare extra-articular manifestation of spondyloarthritis. Its course appears to be independent of joint disease. Its management is specific and should be associated to that of the rheumatologic disorder.

[General practitioner questionnaire concerning upper limb lymphedema after breast cancer]. / Enquête en médecine générale sur les lymphœdèmes du membre supérieur après cancer du sein.

INTRODUCTION: In France, breast cancer is the most frequent cancer in women. Lymphedema, the main complication, is poorly known. The objective of this study was to assess the state of knowledge of upper limb lymphedema (risk factors, complications, treatment) after treatment of breast cancer among general practitioners from the Haute-Normandie and Île-de-France regions. MATERIALS AND METHODS: A cross-sectional study with 23 closed questions was sent by email to 490 practicing doctors. RESULTS: One hundred and sixty-two questionnaires (33%) could be analyzed. Among the participating physicians (men: 55%), 46% were aged over 50 and 75% were from the Haute-Normandie region; 86% of them followed at least 5 women who had breast cancer. Risk factors for lymphedema were: axillary dissection (89%), risk reduction with sentinel lymph node (82%), radiotherapy (81%), mastectomy (45%) and overweight/obesity (42%). For 54% of physicians, lymphedema appeared within 6 months after cancer treatment and was diagnosed upon examination (clinical signs, perimeter measurements) in 78%, without the need for radiological examinations (100%). Physicians with more than 10 years of experience searched for more lymphedema (86% vs. 62%, P<0.0001). Prescriptions included: elastic compression during the day (77%), manual lymphatic drainage (74%), overnight compression (36%) and consultation in a specialized lymphology department (8%). Six percent of physicians had never managed lymphedema and 22% sent patients to an oncologist. Advice given was: prevention of infectious risk (80%), weight loss (42%), avoidance of sports involving the affected limb (22%), and regular arm elevation (14%). Physicians recommended avoiding blood sampling (75%), measuring blood pressure (66%) on the limb ipsilateral to cancer, while 20% did not prohibit any action on the limb. CONCLUSION: Lymphedema knowledge in general practitioners is generally adequate although the number of women followed by each of them was low. It seems necessary to optimize the training of generalists on lymphedema in order to improve patient management.

[Lymphedema in patients treated with sirolimus: 15 cases]. / Lymphœdèmes associés à la prise de sirolimus : à propos de 15 patients.

BACKGROUND: Sirolimus is a mammalian target of rapamycin (mTOR) inhibitor used after organ transplantation and to treat vascular malformations. Among its adverse effects, limb lymphedema has been described. OBJECTIVE: The aim of this study was to analyze the clinical features, lymphoscintigraphy and lymphedema outcome in patients treated with sirolimus. PATIENTS AND METHODS: Monocentric retrospective study from January 2008 to September 2017 analyzing all consecutive patients having lymphedema occurring with sirolimus. RESULTS: Fifteen patients (7 men, 8 women), mean age at the first visit, 56 years (range: 38-76), had a kidney transplant (n=12), liver transplant (n=1), or lymphangioleiomyomatosis (n=2) treated with sirolimus at a mean daily dose of 1.8mg were included. Lymphedema involved one (n=4), or both (n=1) lower limbs, upper limb (n=9), lower limbs and upper limb (n=1). Lymphedema affected the whole limb (n=10), or the distal part (n=5). The median time between lymphedema onset and the beginning of sirolimus was 52 weeks (range: 8-232). Lymphoscintigraphy in 7 patients (lower limb: 3, superior: 4) showed no inguinal or axillary nodal fixation (n=6) or decreased uptake (n=1). Sirolimus was discontinued in 7 cases without lymphedema improvement with a median follow-up of 12 months and maintained in 8 cases. CONCLUSION: Sirolimus is associated with upper and/or lower limb lymphedema, without predominance of sex, and without disappearance after sirolimus discontinuation. Pathophysiological mechanisms remain unclear. Lymphedema management is based on low-stretch bandages and compression.

[Primary intestinal lymphangiectasia (Waldmann's disease)]. / Lymphangiectasies intestinales primitives (maladie de Waldmann).

Primary intestinal lymphangiectasia (PIL), Waldmann's disease, is a rare disorder of unknown etiology characterized by dilated intestinal lacteals leading to lymph leakage into the small-bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. The main symptom is bilateral lower limb edema. Edema may be moderate to severe including pleural effusion, pericarditis or ascites. Protein-losing enteropathy is confirmed by the elevated 24-h stool α1-antitrypsin clearance and diagnosis by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of biopsies. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Several B-cell lymphomas of the gastrointestinal tract or with extra-intestinal localizations were reported in PIL patients. A long-term strictly low-fat diet associated with medium-chain triglyceride and liposoluble vitamin supplementation is the cornerstone of PIL medical management. Octreotide, a somatostatin analog, have been proposed with an inconsistent efficacy in association with diet. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. A prolonged clinical and biological follow-up is recommended.