Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins).

Primary lymphedema is a rare chronic pathology associated with constitutional abnormalities of the lymphatic system. The objective of this French National Diagnosis and Care Protocol (Protocole National de Diagnostic et de Soins; PNDS), based on a critical literature review and multidisciplinary expert consensus, is to provide health professionals with an explanation of the optimal management and care of patients with primary lymphedema. This PNDS, written by consultants at the French National Referral Center for Primary Lymphedema, was published in 2019 ( https://has-sante.fr/upload/docs/application/pdf/2019-02/pnds_lymphoedeme_primaire_final_has.pdf ). Primary lymphedema can be isolated or syndromic (whose manifestations are more complex with a group of symptoms) and mainly affects the lower limbs, or, much more rarely, upper limbs or external genitalia. Women are more frequently affected than men, preferentially young. The diagnosis is clinical, associating mild or non-pitting edema and skin thickening, as confirmed by the Stemmer's sign (impossibility to pinch the skin on the dorsal side or the base of the second toe), which is pathognomonic of lymphedema. Limb lymphoscintigraphy is useful to confirm the diagnosis. Other causes of swelling or edema of the lower limbs must be ruled out, such as lipedema. The main acute lymphedema complication is cellulitis (erysipelas). Functional and psychological repercussions can be major, deteriorating the patient's quality of life. Treatment aims to prevent those complications, reduce the volume with low-stretch bandages, then stabilize it over the long term by exercises and wearing a compression garment. Patient education (or parents of a child) is essential to improve observance.

Short-term Effect and Acceptability of Manual Lymphatic Drainage for Paediatric Limb Lymphoedema: A Prospective Study.

Paediatric lymphoedema (LE) is a rare condition, for which there is little data available regarding treatments. The aim of this study was to assess the short-term effect and acceptability of a 30-min session of manual lymphatic drainage (MLD) in children with well-documented LE of the lower limbs. Fifteen children were included (8 males; median age 11 years). Comparison of the sum of circumference values for the whole limb before and after MLD revealed a slight, but significant, reduction (from a median of 289.8 to 285.5 cm, p = 0.024), but the limb volumes did not decrease significantly (from a median of 4,870.3 to 4,772.3 ml, p = 0.394). Dermal thickness, measured by high-resolution ultrasound, decreased from 1.44 to 1.40 mm (p < 0.001). All children reported improvement in well-being, and found MLD useful. In conclusion, MLD is well accepted by children, but has poor impact on LE swelling. However, it decreases cutaneous oedema by mobilizing the lymph fluid.

[Recognition and initial management of melanoma by general practitioners: A survey in a rural French area with low medical density]. / Reconnaissance et prise en charge initiale du mélanome par des médecins généralistes : enquête dans un département rural à très faible densité médicale.

INTRODUCTION: Cutaneous cancers are very common, easily visible on skin. The general practitioner (GP) is in the front line to manage the lesions. He has to recognize malignancy and refer most often to the specialist for excision, or consider that the lesion is benign and in ambiguous cases, offer clinical monitoring. The current low medical density in the Centre Val de Loire area makes access to general practitioners (GP) and dermatologists harder. The aim of this study was to evaluate GPs' everyday practice in cutaneous cancers screening in general, and particularly in melanoma screening, in a rural region of France. METHODS: We sent a questionnaire on November 24th 2015 to all GPs of the Cher department (204 questionnaires), to assess their knowledge and practice of melanoma. The questionnaire comprised 2 parts: the first part was composed of 23 items with a majority of questions focused on melanoma and the second part consisted of clinical cases including 5 benign cutaneous tumors, 4 melanomas and 1 pigmented basal cell carcinoma. GP's were asked to answer in each case: diagnosis, degree of certainty (from 0 to 10), practical attitude (excision, referral to dermatologist or surgeon or surveillance). Their answers were anonymous. The primary endpoint was whether medical decision regarding each case was appropriate, i.e. removal of malignant lesion or referral for removal, and reassurance or surveillance in benign lesions. RESULTS: The response rate was 35% (72 responses). Thirty-eight GPs (53%) had an appropriate management facing the 5 malignant lesions, 18 GP (25%) had an appropriate management in 4 out of 5 cases. The ABCDE rule was known by 58% of responders, the ugly duckling sign of 19%. GPs did not feel comfortable with melanoma: on a growing scale (from 0=not comfortable at all to 10=very comfortable) the average was 4.2 with a median at 5 (range 0-10). They expressed a strong need for training on this topic: on a scale increasing from 0 to 10, the average was 7.9 for a median to 8 (range 3-10). Knowledge about melanoma risk factors, and the level of ease dealing with screening of melanoma was not significantly associated with a better management. The main difficulties were lack of time and the increasing difficulty of access to dermatologist. Fast and easy access by teledermatology was solicited by 89% of GPs. CONCLUSION: GPs had often appropriate management of skin cancer. The major obstacles to skin's cancer screening were the lack of time and difficult access to dermatologists. The setting-up of an easier access with teledermatology was requested by 89% of responders, and should improve early detection of melanoma.

[Primary lymphedema of limbs]. / Lymphœdèmes primaires des membres.

Limb lymphedema is frequent and not well-known. Clinical classification distinguishes primary lymphedemas due to developmental disorders of the lymphatic system (hereditary or not, sometimes associated with other malformations) and secondary lymphedemas. Primary lymphedema is a lymphedema without a cause to explain lymphatic impairment. It is due to an abnormal lymphangiogenesis in utero. It is often associated with mutation in a gene involved in lymphangiogenesis (FOX C2, VEGFR 3, SOX18, PROX 1…). To assess clinical diagnosis, non-invasive techniques are able to study structure and function of the lymphatic system (mainly isotopic lymphography). Treatment is the complex decongestive therapy which associates manual lymphatic drainage and bandage. Predisposing or precipitating factors have to be treated (particularly streptococcal infections). Surgical treatment has precise and rare indication.