Primary upper-limb lymphoedema

Abstract

Primary ULL appears later in life than LLL, without predominance in either sex. Infectious complications are rare and patients considered the lymphoedema volume stable throughout life.

Paediatric primary lymphoedema: a cohort of 155 children and newborns

“…The interpretation criteria for evaluating lymphatic dysfunction included inguinal lymph node–uptake asymmetry, compared with the contralateral side, ranging from the total absence of visualization to intermediate, diminished lymph node uptake or a normal appearance. Popliteal node visualization was considered an important sign of abnormal lymphatic function, as it suggests rerouting through the deep lymphatic system …”

“…Popliteal node visualization was considered an important sign of abnormal lymphatic function, as it suggests rerouting through the deep lymphatic system. 8,9 Lower-limb lymphoscintigraphy was abnormal (diminished/absent groin lymph node uptake, dermal back flow) in 56 of the 64 (88%) patients tested (Fig. 1d).…”

Primary Upper Limb Lymphedema: Case Report of a Rare Pathology

“…17 Ovarian carcinoma and uterine carcinoma were the most common malignancies. 10,17 Vignes et al, 18 in a review of 60 patients with primary upper limb lymphedema, found similar incidence in males and females, with a mean age at onset of 38.5 years (range 3-82 years). The hand was affected in all patients, the forearm in 55%, and the upper arm in 23%.…”

“…The hand was affected in all patients, the forearm in 55%, and the upper arm in 23%. 18 Twenty-one patients (35%) had associated lower limb lymphedema. Patients were followed for a median period of 103 months, and lymphedema was stable in 95%.…”

Measurement of skin thickness and skin elasticity to evaluate the effectiveness of intensive decongestive treatment in patients with lymphoedema: a prospective study

“…Lymphoedema is a particular type of oedema caused by a congenital or acquired deficiency of the lymphatic system, resulting in an accumulation of protein‐rich fluid in the dermis and hypodermis . Primary lymphoedemas are a heterogeneous group of genetic diseases with known mutations of the VEGFR3 gene in the case of Milroy's disease and of the FOXC2 gene in lymphoedema associated with distichiasis , resulting in functional abnormality of the lymphatic system.…”