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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Orphanet: Eritrodermia ictiosiforme bolhosa congenita

Palmoplantar involvement is seen in some patients. Ultrasonography can detect the collodion membrane.

Additional information Further information on this disease Classification s 2 Gene s 2 Clinical signs and symptoms Publications in PubMed Other website s 5. NIPAL4 likely encodes a membrane receptor. Immunohistochemistry using antibodies directed against TGase 1 or Ictiosifrme enzyme activity measurement is available in some centers.

The clinical picture can also change over time and in response to treatment. All articles are subjected to a rigorous process of revision in pairs, and careful editing for literary and scientific style.


eritrodermia ictiosiforme congénita – English Translation – Word Magic Spanish-English Dictionary

Subscribe to our Newsletter. Summary and related texts. Prognosis The severity of the disease is variable. Epidermolytic ichthyosis EI is a rare keratinopathic ichthyosis KPI; see this termthat is characterized by a blistering phenotype at birth which progressively becomes hyperkeratotic. Health care resources for this disease Expert centres 78 Diagnostic tests 24 Patient organisations 27 Orphan drug s 3. Other additional clinical features can be observed in variable degrees of severity: Are you a health professional able to prescribe or dispense drugs?

For all other comments, please send your icitosiforme via contact us. Genetic counseling The disease is transmitted as an autosomal recessive trait.

Emollients are often used but their efficacy is limited. Management and treatment Management is based on daily applications of emollients. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal’s impact.

For all other comments, please send your remarks via contact us. Other features may include hypohidrosis, scalp cognenita and nail dystrophy.


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The life expectancy is normal. Differential diagnosis At birth, differential diagnoses include other causes of neonatal erythroderma e.

This item has received. Genetic counseling should be offered to affected families. From Monday to Friday from 9 a. July Pages CiteScore measures average citations received per document published. The rest are autosomal dominant cases, more rarely autosomal recessive.

Subscriber If you already have your login eritroderrmia, please click here. The diagnosis is based on the clinical appearance of the skin. Check this box if you wish to receive a copy of your message.

Skin is often itchy and smelly, and skin infection may occur. Go to the members area of the website of the AEDV, https: