Erythrokeratodermia variabilis (also known as "erythrokeratodermia figurata variabilis", "keratosis extremitatum progrediens", "keratosis palmoplantaris transgrediens et progrediens",: 509  "Mendes da Costa syndrome", "Mendes da Costa type erythrokeratodermia", and "progressive symmetric erythrokeratoderma") is a rare autosomal dominant disorder that usually presents at birth or during the first year of life. To date, it is thought to be caused by mutations in genes encoding for connexin channels proteins in the epidermis, leading to the misregulation of homeostasis in keratinocytes.One type is characterized by generalized, persistent, brown hyperkeratosis with accentuated skin markings, while a second type is localized, with involvement that is limited in extent and characterized by sharply demarcated, hyperkeratotic plaques. : 565 It can be associated with GJB3 and GJB4. It was characterized in 1925. See also
List of cutaneous conditions


== References ==
Management
After initial diagnosis of oligohydramnios has been made, the next step is to perform a thorough history and physical exam, followed by diagnostic testing if indicated. Retaking a maternal and family history and performing a physical exam can point to maternal conditions or medications that might be causing the oligohydramnios. Premature prelabor rupture of membranes or prelabor rupture of membranes is ruled out with a nitrizine test, evidence of ferning, or evidence pooling of liquid in the cervix. Sonographic evaluation of the fetus is done to identify fetal anomalies, aneuploidy, fetal growth restriction, or placental abnormalities. The National Institute of Health recommends detailed documentation of certain fetal organs that are most likely to be involved such as the kidneys, bladder, and the umbilical cord. If the lack of amniotic fluid prevents accurate visualization on ultraosund, MRI imaging can be considered. Genetic testing can be useful if fetal anomalies are documented on imaging. An elevated maternal serum alpha fetal protein (MSAFP) can indicate leaking amniotic fluid due to damage to fetal membranes or the placenta. This is associated with a poor prognosis. A maternal blood test or amniotic fluid test can be performed if suspicion of a TORCH infection is high. Increasing amniotic fluid
There is no way to permanently increase the volume of amniotic fluid, but it can be temporarily increased to allow for a complete anatomy scan of the fetus on ultrasound.One way to achieve this is through an amnioinfusion, which is the insertion of 200mL of saline into the amniotic sac.