|1.||Hearing Loss (Hearing Impairment)
|2.||Deafness (Deaf Mutism)
|3.||Peripheral Nervous System Diseases (PNS Diseases)
|5.||Malignant Atrophic Papulosis
|1.||Pan, Qian: 4 articles (11/2015 - 06/2005)|
|2.||Chi, Jingwei: 2 articles (11/2015 - 01/2012)|
|3.||Liu, Mujun: 2 articles (11/2015 - 01/2012)|
|4.||Wang, Danling: 2 articles (11/2015 - 01/2012)|
|5.||Tang, Chengyuan: 2 articles (11/2015 - 01/2012)|
|6.||Zhang, Zhuohua: 2 articles (11/2015 - 01/2012)|
|7.||Liu, Yu: 2 articles (08/2005 - 06/2005)|
|8.||Xia, Kun: 2 articles (08/2005 - 06/2005)|
|9.||Cai, Fang: 2 articles (08/2005 - 06/2005)|
|10.||Hohl, Daniel: 2 articles (12/2003 - 07/2002)|
07/01/2014 - "Both low-dose arotinoid ethylester and acitretin are effective in the treatment of familial erythrokeratodermia variabilis."
11/01/2002 - "Acitretin for erythrokeratodermia variabilis in a 9-year-old girl."
01/01/1990 - "A patient with erythrokeratodermia variabilis (Mendes da Costa's disease) is presented, and the clinical and histological response to acitretin is described. "
01/01/1990 - "Acitretin in the treatment of erythrokeratodermia variabilis."
11/01/2008 - "Erythrokeratodermia variabilis: successful palliative treatment with acitretin."
10/15/1989 - "[A case of erythrokeratodermia figurata variabilis successfully treated with tigason]."
03/01/1979 - "[Erythrokeratodermia figurata variabilis Mendes da Costa: successful treatment using an oral aromatic retinoid (ro-10 9359)]."
01/01/1987 - "Immunohistochemical and ultrastructural study of the epidermis was performed in a 53-year-old female with erythrokeratodermia variabilis (EKV) before and after treatment with the aromatic retinoid Etretinate (RO 10-9359). "
01/01/1980 - "In 5 children ranging in age from 8 to 12 years, treatment with Ro 10-9359 for either psoriasis or erythrokeratodermia variabilis for periods of between 11 and 17 months did not cause marked growth retardation and gave excellent therapeutic results."
|3.||human GJB3 proteinIBA
11/01/2015 - "Mutations in connexin-31 (Cx31) are associated with multiple human diseases, including familial erythrokeratodermia variabilis (EKV). "
04/01/2012 - "A case of erythrokeratodermia variabilis with connexin 31 gene mutation (Cx31F137L)."
01/01/2012 - "Mutations in Connexin-31 (Cx31) are associated with multiple human diseases including erythrokeratodermia variabilis (EKV). "
03/01/2004 - "A novel recessive connexin 31 (GJB3) mutation in a case of erythrokeratodermia variabilis."
12/15/2003 - "We recently identified mutations in either GJB3 or GJB4 genes, encoding respectively connexin 31 (Cx31) or 30.3 (Cx30.3), as causally involved in erythrokeratodermia variabilis (EKV), a mostly autosomal dominant disorder of keratinization. "
06/01/2015 - "Erythrokeratodermia variabilis et progressiva (EKVP) is a genodermatosis with clinical and genetic heterogeneity, most often transmitted in an autosomal dominant manner, caused by mutations in GJB3 and GJB4 genes encoding connexins (Cx)31 and 30.3, respectively. "
05/01/2005 - "[Erythrokeratodermia variabilis (EKV)--a disorder due to altered epidermal expression of gap junction proteins]."
11/01/1997 - "There is evidence, however, that other epidermally expressed connexins cluster in this region, and one may yet be determined to play a role in the pathogenesis of erythrokeratodermia variabilis."
10/01/2003 - "Her clinical presentation, history, and histopathologic analysis were consistent with erythrokeratodermia variabilis, which is a genodermatosis linked to mutations in the gene encoding for the gap-junction protein, connexin 31."
01/01/2004 - "Mutations in the GJB3 gene, encoding the gap junction protein connexin 31 (Cx31), have been pathogenically linked to erythrokeratodermia variabilis and nonsyndromic autosomal recessive or dominant hereditary hearing impairment. "
03/27/2013 - "Novel mutation in GJB4 gene (connexin 30.3) in a family with erythrokeratodermia variabilis."
03/01/2012 - "Erythrokeratodermia variabilis: report of two cases and a novel missense variant in GJB4 encoding connexin 30.3."
11/01/2000 - "Mutation in the gene for connexin 30.3 in a family with erythrokeratodermia variabilis."
12/15/2003 - "Molecular interaction of connexin 30.3 and connexin 31 suggests a dominant-negative mechanism associated with erythrokeratodermia variabilis."
01/01/2006 - "Erythrokeratodermia variabilis with adult onset: report of a sporadic case unresponsive to systemic retinoids."
03/01/1980 - "Treatment of erythrokeratodermia variabilis with oral synthetic retinoids."
01/01/1990 - "In comparison with the other retinoids available so far, acitretin is the derivative of first choice in the treatment of erythrokeratodermia variabilis Mendes da Costa."
|7.||Vitamin A (Retinol)FDA LinkGeneric
06/01/2011 - "PSEK shares many clinical features with erythrokeratodermia variabilis (EKV), which is associated with mutations in genes coding for gap junction beta (GJB) 3 and 4. A mutation in the loricrin gene (LOR) was found in patients with PSEK, who were members of a family with Vohwinkel syndrome. "
07/01/2002 - "The differential diagnoses of the following dermatoses with an erythematous and a hyperkeratotic component are discussed: erythrokeratodermia variabilis (Mendes da Costa), progressive symmetric erythrokeratoderma (Gottron), loricrin keratoderma, erythrokeratoderma en cocardes (Degos), Netherton syndrome, keratitis-ichthyosis-deafness (KID) syndrome, erythrokeratolysis hiemalis (Oudtshoorn disease), and nonbullous congenital ichthyosiform erythroderma."
|9.||Isotretinoin (Accutane)FDA LinkGeneric
|10.||Staphylococcal Protein A (A, Protein)IBA
|3.||Palliative Care (Palliative Medicine)