Eritrodermia ictiosiforme congénita ampollosa: a propósito de un caso. Bullous congenital ichthyosiform erythroderma: a case report. Nuria Romero Maldonado . Request PDF on ResearchGate | Eritrodermia ictiosiforme congénita no ampollosa asociada a pénfigo vegetante. Presentación de caso | Background: The. Request PDF on ResearchGate | On Jan 1, , J.R. Antônio and others published Eritrodermia ictiosiforme congênita bolhosa.
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Summary and related texts. The clinical picture can also change over time and in response to treatment. NIPAL4 likely encodes a membrane receptor. Other additional clinical features can be observed in variable degrees of severity: Prognosis Prognosis is variable, ranging from ictiosigorme to severe especially during the neonatal period due to the risk of sepsis.
Histological aspect of the skin is not specific. Diagnostic methods Diagnosis is based on the clinical picture and eritfodermia histological examination of skin lesion biopsies showing hyperkeratosis with orthokeratosis, hypergranulosis, and cytolysis in the upper stratum spinosum and granular layers epidermolytic hyperkeratosis.
The disease is transmitted as an autosomal recessive trait. Detailed information Article for general public Svenska If you are a member of the AEDV: At birth, differential diagnosis includes toxic epidermal necrolysis, inherited epidermolysis bullosa, incontinentia pigmenti or herpetic infection, while in congeniya stages it includes other KPIs such as superficial EI and ichthyosis congwnita of Curth Macklin see these terms.
The life expectancy is normal. Pemphigus Vegetans in the Inguinal Folds. CIE is a genetically heterogeneous disease.
Over time, hyperkeratosis worsens and blister formation decreases but may still occur following skin trauma or during summer. Prenatal diagnosis is based on DNA analysis of amniocentesis and chorion villus sampling materials.
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Molecular testing is possible but is not available in general practice. CiteScore measures average citations received per document published. Additional information Further information on this disease Classification s 5 Gene s 7 Clinical signs and symptoms Other website s 2.
Clinical description Since birth, patients present with fine white-grayish scales of various sizes associated with erythroderma. Etiology CIE is a genetically heterogeneous disease. Diagnosis is based on the clinical picture and on histological examination of skin lesion biopsies showing hyperkeratosis with orthokeratosis, hypergranulosis, and cytolysis in the upper stratum spinosum and granular layers epidermolytic hyperkeratosis. Clinical description Infants present at birth, or shortly after, with generalized erythroderma, severe blistering, mild scaling, and superficial skin erosions at sites of minor trauma and flexural areas.
Most cases are sporadic. Antenatal diagnosis Prenatal diagnosis is based on DNA analysis of amniocentesis and chorion villus sampling materials.
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Other search option s Alphabetical list. For all other comments, please send your remarks via contact us. Specialised Social Services Eurordis directory. Topical keratolytics or oral acitretin for severe forms can improve hyperkeratotic lesions, but are associated with adverse effects such as skin fragility and worsening of blistering. Bullous congenital ichthyosiform erythroderma: Growth failure may be seen in severe cases. Subscriber If you already have your login data, please click here.
Palmoplantar involvement is seen in some patients. In the year has been indexed in the Medlinedatabase, and has become a vehicle for expressing the most current Spanish eritroderima and modern. The exact prevalence of EI is unknown.
eritrodermia ictiosiforme congénita – English Translation – Word Magic Spanish-English Dictionary
The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. The material congenitq in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.
InfancyNeonatal ICD Since birth, patients present with fine white-grayish scales of various sizes associated with erythroderma. At birth, differential diagnoses include other causes of neonatal erythroderma e.
Repositório do Hospital Prof. Doutor Fernando Fonseca: Eritrodermia ictiosiforme congénita
Antiseptic washes reduce the bacterial colonization and body odor. Management is based on daily applications of emollients. A clinical variant of EI, annular EI see this termshows an annular distribution of polycyclic erythematous scales that generally develop on the trunk and extremities and that tend to resolve. Are you a health professional able to prescribe congrnita dispense drugs?
Genetic counseling Most cases are sporadic. Continuing navigation will be ictiosifkrme as acceptance of this use. Management and treatment Management is based on daily applications of emollients. Yellow-brown hyperkeratotic plaques, often associated with mild background erythroderma, develop later, usually in the first months of life.