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Disease Information for Lymphedema, hereditary

Clinical Manifestations

Signs & Symptoms

Edema Children

Bilateral ankle swelling

Bilateral leg edema/swelling

Hypertrophic lymphatics/subcutaneous/signs

Indurated lymphatics/extremity/regional

Edema of Lower Extremities

Edema, lower extremity (leg) , unilateral

Foot Swelling

Leg/lower extremity pains

Lymphedema of lower extremities

Lymphedema of one leg

Swelling one extremity/limb

Swollen Limb

Thigh/Leg Swelling Unilateral Severe

Unilateral leg swelling

Unilateral Leg swelling in Children

Neonatal edema/anasarca

Disease Progression

Course/Chronic only

Demographics & Risk Factors

Population Group

Child

Population/Pediatrics population

Sex & Age Groups

Population/Child

Population/Child-Infant Only

Population/Children/all

Associated Diseases & Rule outs

Rule Outs

Elephantiasis

Associated Disease & Complications

Cellulitis, leg/ankles

Edema

Hemihypertrophy

Lymphedema, hereditary

Disease Mechanism & Classification

Class

CLASS/Pediatric disorders (ex)

CLASS/Dermatologic/Subcutaneous (category)

CLASS/Lymphatics/channels disorders (ex)

CLASS/Lymphatics/lymph (category)

CLASS/Extremities/Digits/Hand feet disorder (category)

CLASS/Muscle/tendon/extremities (category)

Pathophysiology

Pathophysiology/Edematous process

Pathophysiology/Gene locus 16q24.3

Pathophysiology/Gene locus 5q35.3

Pathophysiology/Gene locus Chromosome 16

Pathophysiology/Gene locus Chromosome 5

Pathophysiology/Gene Locus Identified/OMIM database

Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)

Process

PROCESS/Congenital/developmental (category)

PROCESS/Hereditofamilial (category)

Synonyms

Synonym

Congenital lymphedema, Congenital lymphoedema, Disease Milroy, Disease Milroy's, edema Milroy, Hereditary lymphedema type I, Hereditary lymphedema type I (disorder), Hereditary lymphoedema type I, Infantile lymphedema (praecox), Infantile lymphedema(praecox), Infantile lymphoedema (praecox), Infantile lymphoedema(praecox), Lymphedema congenital, LYMPHEDEMA EARLY ONSET, LYMPHEDEMA HEREDITARY I, Lymphoedema congenital, Milroy, MILROY DIS, MILROY DISEASE, Milroy edema, Milroy lymphedema, Milroy lymphoedema, MILROYS DIS, Milroys Disease, Milroy's Disease, Milroy's disease (disorder), Nonne Milroy, NONNE MILROY LYMPHEDEMA, Nonne Milroy lymphoedema, NONNE MILROY MEIGE SYNDROME, PCL, Primary (congenit )lymphedema, Primary (congenit )lymphoedema, Primary (congenital) lymphedema, Primary (congenital) lymphoedema, PRIMARY CONGENITAL LYMPHEDEMA, Primary congenital lymphoedema, Synonym/Milroy's disease, Synonym/Nonne-Milroy-Meige syndrome

Definition

Meige"s syndrome I; here is some confusion among authors about what is what with Meige’s syndrome, Milroy’s syndrome, Nonne’s syndrome and Nonne-Milroy-Meige syndrome; According to one author, hereditary lymphoedema type II is the Meige syndrome; hereditary lymphoedema I is the Nonne-Milroy syndrome, also commonly referred to as Milroy disease; Some authors still combine these into a single syndrome, the Nonne-Milroy-Meige syndrome;Meige"s disease;

Milroy"s disease; Nonne’s syndrome; Nonne-Milroy syndrome

Meige"s syndrome I; Chronic hereditary trophoedema, chronic hereditary lymphoedema, congenital elephantiasis, congenital lymphedema, elephantiasis congenita hereditaria (Nonne); familial hereditary oedema, hereditary lymphedema I (Nonne-Milroy); hereditary lymphedema II (Meige), hereditary tropholymphoedema syndrome; idiopathic hereditary lymphedema, lymphoedema; lymphedema, early onset type; trophoedema hereditarium (chronicum extremitatum inferiorum); tropholymphoedema;

Chronic familial lymphoedema of the limbs, which manifests with pittings and brawny swellings (lymphoedema) of the ankles and shins; Often associated with inflammation and various defects, including distichiasis, extradural cysts, vertebral anomalies, cerebrovascular malformations, yellow nails, and sensorineural hearing loss; Congenital or onset in first or second decade of life; It is due to inadequate lymph flow through an anatomically abnormal lymphatic system; Prevalent in females (70-80%); Pain, sometimes severe, is a common feature; Autosomal dominant inheritance with variable expressivity; There is a lot of confusion about the correct term for this condition; According to some authors the term Nonne-Milroy-Meige syndrome is obsolete, as this is really two distinct entities, Hereditary lymphedema I (Nonne-Milroy syndrome) with onset at birth to 35 years of age; and hereditary lymphedema type II (Meige syndrome) with onset after 35 years; -------------

[whonamedit_com 2005]---------------

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External Links Related to Lymphedema, hereditary

PubMed (National Library of Medicine)

NGC (National Guideline Clearinghouse)

Medscape (eMedicine)

Harrison's Online (accessmedicine)

NEJM (The New England Journal of Medicine)

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