Congenital lymphedema

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Congenital lymphedema is a blockage of fluid in the developing fetal lymphatic system. It is diagnosed by a nuchal scan. Presence of this condition is also associated with congenital heart defect.^[1]

[edit] See also

[edit] References

^ "Relationship Between Nuchal Translucency Thickness and Prevalence of Major Cardiac Defects in Fetuses With Normal Karyotype", by Atzei, A; Gajewska, K; Huggon, I C.; Allan, L; Nicolaides, K H. Obstetrical and Gynecological Survey. January 2006. Volume 61, Issue 1, pages 8-10.

Congenital malformations and deformations of integument / skin disease (Q80–Q82, 757.0–757.3)

Congenital ichthyosis/
erythrokeratodermia

AD	Ichthyosis vulgaris

AR	Congenital ichthyosiform erythroderma: Epidermolytic hyperkeratosis · Lamellar ichthyosis (Harlequin type ichthyosis) Netherton syndrome · Zunich–Kaye syndrome · Sjögren–Larsson syndrome

XR	X-linked ichthyosis

Ungrouped	Ichthyosis bullosa of Siemens · Ichthyosis follicularis · Ichthyosis prematurity syndrome · Ichthyosis–sclerosing cholangitis syndrome · Nonbullous congenital ichthyosiform erythroderma · Ichthyosis linearis circumflexa · Ichthyosis hystrix

EB
and related

EBS (EBS-K, EBS-WC, EBS-DM, EBS-OG, EBS-MD, EBS-MP)

JEB (JEB-H, Mitis, Generalized atrophic, JEB-PA)

DEB (DDEB, RDEB)

related: Costello syndrome · Kindler syndrome · Laryngoonychocutaneous syndrome · Skin fragility syndrome ·

Ectodermal dysplasia

Naegeli syndrome/Dermatopathia pigmentosa reticularis · Hay–Wells syndrome · Hypohidrotic ectodermal dysplasia · Focal dermal hypoplasia · Ellis–van Creveld syndrome · Rapp–Hodgkin syndrome/Hay–Wells syndrome

Elastic/Connective

Ehlers–Danlos syndrome · Cutis laxa (Gerodermia osteodysplastica) · Popliteal pterygium syndrome · Pseudoxanthoma elasticum · Van Der Woude syndrome

Hyperkeratosis/
keratinopathy

PPK	diffuse: Diffuse epidermolytic palmoplantar keratoderma • Diffuse nonepidermolytic palmoplantar keratoderma • Palmoplantar keratoderma of Sybert • Mal de Meleda • syndromic (connexin (Bart–Pumphrey syndrome • Clouston's hidrotic ectodermal dysplasia • Vohwinkel syndrome) • Corneodermatoosseous syndrome • plakoglobin (Naxos syndrome) • Scleroatrophic syndrome of Huriez • Olmsted syndrome • Cathepsin C (Papillon–Lefèvre syndrome • Haim–Munk syndrome) • Camisa disease focal: Focal palmoplantar keratoderma with oral mucosal hyperkeratosis • Focal palmoplantar and gingival keratosis • Howel–Evans syndrome • Pachyonychia congenita (Pachyonychia congenita type I • Pachyonychia congenita type II) • Striate palmoplantar keratoderma • Tyrosinemia type II) punctate: Acrokeratoelastoidosis of Costa • Focal acral hyperkeratosis • Keratosis punctata palmaris et plantaris • Keratosis punctata of the palmar creases • Schöpf–Schulz–Passarge syndrome • Porokeratosis plantaris discreta • Spiny keratoderma ungrouped: Palmoplantar keratoderma and spastic paraplegia • desmoplakin (Carvajal syndrome) • connexin (Erythrokeratodermia variabilis • HID/KID)

Other	Meleda disease · Keratosis pilaris · ATP2A2 (Darier's disease) · Dyskeratosis congenita · Lelis syndrome Dyskeratosis congenita · Keratolytic winter erythema · Keratosis follicularis spinulosa decalvans · Keratosis linearis with ichthyosis congenital and sclerosing keratoderma syndrome · Keratosis pilaris atrophicans faciei · Keratosis pilaris

Other

cadherin (EEM syndrome) · immune system (Hereditary lymphedema, Mastocytosis/Urticaria pigmentosa) · Hailey–Hailey

see also Template:Congenital malformations and deformations of skin appendages, Template:Phakomatoses, Template:Pigmentation disorders, Template:DNA replication and repair-deficiency disorder

Developmental
anomalies

Midline	Dermoid cyst · Encephalocele · Nasal glioma · PHACE association · Sinus pericranii

Nevus	Capillary hemangioma · Port-wine stain (Nevus flammeus nuchae)

Other/ungrouped	Aplasia cutis congenita · Amniotic band syndrome · Branchial cyst · Cavernous venous malformation Accessory nail of the fifth toe · Bronchogenic cyst · Congenital cartilaginous rest of the neck · Congenital hypertrophy of the lateral fold of the hallux · Congenital lip pit · Congenital malformations of the dermatoglyphs · Congenital preauricular fistula · Congenital smooth muscle hamartoma · Cystic lymphatic malformation · Median raphe cyst · Melanotic neuroectodermal tumor of infancy · Mongolian spot · Nasolacrimal duct cyst · Omphalomesenteric duct cyst · Poland anomaly · Rapidly involuting congenital hemangioma · Rosenthal–Kloepfer syndrome · Skin dimple · Superficial lymphatic malformation · Thyroglossal duct cyst · Verrucous vascular malformation · Birthmark

M: INT, SF, LCT

noco(i/b/d/q/u/r/p/m/k/v/f)/cong/tumr(n/e/d), sysi/epon

proc, drug (D2/3/4/5/8/11)

Pathology: lymphatic disease: Lymphatic vessel disease (I88–I89, 457)

Lymphangiectasia

Lymphedema: Primary lymphedema (Congenital lymphedema, Lymphedema praecox, Lymphedema tarda, Lymphedema–distichiasis syndrome, Milroy's disease) · Secondary lymphedema (Bullous lymphedema, Factitial lymphedema, Postinflammatory lymphedema, Postmastectomy lymphangiosarcoma)

Waldmann disease

anat(a:h/u/t/a/l,v:h/u/t/a/l)/phys/devp/cell/prot

noco/syva/cong/lyvd/tumr, sysi/epon, injr

proc, drug(C2s+n/3/4/5/7/8/9)

Pathology: hemodynamics

Decreases

Thrombus/thrombosis	Renal vein thrombosis

Ischemia	Brain ischemia · Ischaemic heart disease · Ischemic colitis/Mesenteric ischemia

Infarction	general: Anemic infarct · Hemorrhagic infarct regional: Myocardial infarction · Cerebral infarction · Splenic infarction · Limb infarction

Increases

Hemorrhage	general Bruise/Hematoma: Petechia · Purpura · Ecchymosis regional: head (Epistaxis, Hemoptysis, Intracranial hemorrhage, Hyphema, Subconjunctival hemorrhage) · torso (Hemothorax, Hemopericardium, Pulmonary hematoma) · abdomen (Gastrointestinal bleeding, Haemobilia, Hemoperitoneum, Hematocele, Hematosalpinx ) · joint (Hemarthrosis)

Edema	general: Anasarca · Angioedema/Lymphedema · Exudate/Transudate regional: Cerebral edema · Pulmonary edema · Hydrothorax · Ascites/hydroperitoneum · Hydrosalpinx

Other	Hyperemia

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