U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Body skin hyperlaxity due to vitamin K-dependent coagulation factor deficiency

MedGen UID:
332067
Concept ID:
C1835813
Disease or Syndrome
Synonyms: Pseudoxanthoma elasticum-like disorder with multiple coagulation factor deficiency; PXE-LIKE DISORDER WITH MULTIPLE COAGULATION FACTOR DEFICIENCY
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
Gene (location): GGCX (2p11.2)
 
Monarch Initiative: MONDO:0012570
OMIM®: 610842
Orphanet: ORPHA91135

Definition

Body skin hyperlaxity due to vitamin K-dependent coagulation factor deficiency is a very rare genetic skin disease characterized by severe skin laxity affecting the trunk and limbs. [from ORDO]

Clinical features

From HPO
Atherosclerosis
MedGen UID:
13948
Concept ID:
C0004153
Disease or Syndrome
A condition characterized by patchy atheromas or atherosclerotic plaques which develop in the walls of medium-sized and large arteries and can lead to arterial stenosis with reduced or blocked blood flow.
Epistaxis
MedGen UID:
4996
Concept ID:
C0014591
Pathologic Function
Epistaxis, or nosebleed, refers to a hemorrhage localized in the nose.
Prolonged prothrombin time
MedGen UID:
208879
Concept ID:
C0853225
Finding
Increased time to coagulation in the prothrombin time test, which is a measure of the extrinsic pathway of coagulation. The results of the prothrombin time test are often expressed in terms of the International normalized ratio (INR), which is calculated as a ratio of the patient's prothrombin time (PT) to a control PT standardized for the potency of the thromboplastin reagent developed by the World Health Organization (WHO) using the formula
Abnormal bleeding
MedGen UID:
264316
Concept ID:
C1458140
Pathologic Function
An abnormal susceptibility to bleeding, often referred to as a bleeding diathesis. A bleeding diathesis may be related to vascular, platelet and coagulation defects.
Reduced factor IX activity
MedGen UID:
868754
Concept ID:
C4023159
Finding
Decreased activity of coagulation factor IX. Factor IX, which itself is activated by factor Xa or factor VIIa to form factor IXa, activates factor X into factor Xa.
Reduced factor X activity
MedGen UID:
892449
Concept ID:
C4024702
Finding
Reduced activity of coagulation factor X. The extrinsic and intrinsic pathways converge at factor X (fX). The extrinsic pathway activates fX by means of d factor VII with its cofactor, tissue factor. The intrinsic pathway activates fX by means of the tenase complex (Ca2+ and factors VIIIa, IXa and X) on the surface of activated platelets. Factor Xa in turn activates prothrombin (factor II) to thrombin (factor IIa).
Reduced factor VII activity
MedGen UID:
892851
Concept ID:
C4024722
Finding
Reduced activity of coagulation factor VII. Factor VII is part of the extrinsic coagulation pathway, which is initiated at the site of injury in response to the release of tissue factor (fIII). Tissue factor and activated factor VII catalyze the activation of factor X.
Cutis laxa
MedGen UID:
8206
Concept ID:
C0010495
Disease or Syndrome
Wrinkled, redundant, inelastic and sagging skin.
Yellow papule
MedGen UID:
1373750
Concept ID:
C2033396
Finding
A papule with yellow color.
Angioid streaks
MedGen UID:
1541
Concept ID:
C0002982
Disease or Syndrome
Angioid streaks are irregular tapering linear breaks in the Bruch membrane that typically emanate from the optic disk (summary by Karacorlu et al., 2002).
Retinal peau d'orange
MedGen UID:
1713761
Concept ID:
C5398025
Finding
A pebbly orange appearance of the fundus that is said to resemble the skin of an orange.

Suggested Reading

PubMed

Uitto J, Bercovitch L, Terry SF, Terry PF
Am J Med Genet A 2011 Jul;155A(7):1517-26. Epub 2011 Jun 10 doi: 10.1002/ajmg.a.34067. PMID: 21671388Free PMC Article

Supplemental Content

Recent activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...