Besides presenting the case, we review the literature on this rare disorder of keratinization. [ncbi.nlm.nih.gov]

Keratohyaline granules are present along the rudimentary keratin skeleton at the periphery of the cytoplasm (arrowheads). [derm101.com]

It usually presents at birth as erythroderma (fig 1). [adc.bmj.com]

The timing of onset is perhaps the most important feature, as erythroderma present at birth has fewer potential causes than later presentation. [dovepress.com]

Clinical features which were not always present in every patient were considered as minor criteria. [link.springer.com]

• Ear Deformity

  Indeed, among 40 previously reported IWC patients, 7 showed both ear deformities and mammillae hypoplasia [ 17, 19 ], whereas 6 presented ear deformities [ 7, 8, 18 ] or mammillae hypoplasia [ 3, 12, 19 ] alone (Table 1 ). [link.springer.com]

• Hypertrichosis

  They have hypertrichosis. B. [emedicalhub.com]

  Affiliated tissues include skin, and related phenotypes are hypertrichosis and clubbing Genetics Home Reference : 26 Ichthyosis with confetti is a disorder of the skin. [malacards.org]

  Clinical features : Imported from Human Phenotype Ontology (HPO) Abnormality of limbs Clubbing Hereditary palmoplantar keratoderma Abnormality of the immune system Erythroderma Abnormality of the integument Erythroderma Hereditary palmoplantar keratoderma Hypertrichosis [familydiagnosis.com]

  Hypertrichosis is a frequent and characteristic IWC sign [ 10, 11, 14, 17, 19 ]. [link.springer.com]

  Another peculiar clinical feature is a remarkable hypertrichosis. At the ultrastructural level, perinuclear deposits of filamentous material in vacuolized keratinocytes of the upper epidermis, pathognomonic for CRIE, were demonstrated. [ncbi.nlm.nih.gov]

• Epistaxis

  Acute adverse effects of systemic retinoids include skin irritation, fragility and tenderness, mucocutanous toxicities (in particular cheilitis, epistaxis, eye irritation), hair loss, and laboratory abnormalities in blood cell counts, transaminases and [link.springer.com]

• Hyperreflexia

  However, peripheral hyperreflexia has been detected by neurologic examination in four patients [ 15 ] and psychomotor retardation has been reported in one case [ 17 ]. [link.springer.com]

• Neglect

  Ichthyosis variegata: a new name for a neglected disease. J Am Acad Dermatol. 1997;36:500. CrossRef PubMed 3. Marghescu S, Anton-Lamprecht I, Rudolph PO, Kaste R. Congenital reticular ichthyosiform erythroderma. Hautarzt. 1984;35:522–9. PubMed 4. [springermedizin.de]

• Nystagmus

  Additional but rarely reported signs of eye involvement are strabismus and nystagmus [ 19 ]. Finger length relative to palms may be decreased [ 19 ]. [link.springer.com]

Once the erythroderma is being managed, further workup for underlying pathology is imperative as the therapies vary depending on the underlying disease processes. B. Anticipated length of Stay. [cancertherapyadvisor.com]

Initial diagnostic workup is included in Table 2. Figure 5 Algorithm for diagnosis of the erythrodermic infant. [dovepress.com]

Controlling symptoms with emollients is the goal of treatment for ichthyosis since it has no cure. [casereports.bmj.com]

Effect of topical tazarotene in the treatment of congenital ichthyoses. Br J Dermatol. 1999;141:642–6. CrossRef PubMed 38. Craiglow BG, Choate KA, Milstone LM. Topical tazarotene for the treatment of ectropion in ichthyosis. [springermedizin.de]

New sections on treatment that highlight recent treatment trials and guidelines. Clinical descriptions updated. Brand new illustrations incorporated throughout. 14,000 new references. [books.google.com]

Treatment of the underlying disease process decreases the episodes of erythroderma and may correct the nutritional and fluid imbalances. Treatment of underlying dermatologic processes is complex and is best done by the dermatologist. E. [cancertherapyadvisor.com]

The patient was discharged on the same treatment, with advice for regular checkup of blood counts. [docslide.com.br]

Prognosis and Patient Counseling. Prognosis is good but depends somewhat on underlying disease processes. VI. Patient Safety and Quality Measures. A. Core Indicator Standards and Documentation. NA B. [cancertherapyadvisor.com]

In conclusion, erythroderma is a complex multifactorial dermatosis, the prognosis of which depends on the causative agent. [ncbi.nlm.nih.gov]

The course, prognosis, and management strategies also vary depending on the cause of erythroderma; hence, an accurate diagnosis is essential in minimizing associated morbidity and mortality... [readbyqxmd.com]

A severe burn-like erythema has been described in this group and is associated with a worse prognosis. [dovepress.com]

Prognosis IWC, as most other heritable ichthyoses, is a condition that requires significant attention in the neonatal period. [link.springer.com]

[…] are rarer etiologies, but important to recognize early to prevent harmful sequelae. [dovepress.com]

Elimination of infectious etiologies and the temporal association of new drug ingestion, and absence of hypotension would support this etiology. [cancertherapyadvisor.com]

Etiology and pathogenesis X-linked ichthyosis results from mutations of the STS gene encoding steroid sulfatase (arylsulfatase C), particularly deletions (90% of patients). [clinicalgate.com]

Erythroderma: a clinical-etiological study of 82 cases. European Journal of Dermatology. 2010;20(3):373–377. [PubMed] [Google Scholar] 7. Altiner A, Chu J, Patel R, Latkowski J-A, Schaffer J, Sanders S. Erythroderma of unknown etiology. [ncbi.nlm.nih.gov]

[…] palmoplantaris striata Superficial epidermolytic ichthyosis Synonym(s): - CRIE - IWC - Ichthyosis variegata - Ichthyosis with confetti Classification (Orphanet): - Rare genetic disease - Rare skin disease Classification (ICD10): (no data available) Epidemiological [csbg.cnb.csic.es]

"Blood Pressure" [SH] (epidemiology AND humans) [SH] SI Secondary Source ID The SI field identifies secondary source databanks and accession numbers, e.g., GenBank, GEO, PubChem, ClinicalTrials.gov, ISRCTN. [biomedsearch.com]

Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatol. 2006;45:614-20. [ Links ] 21 Akiyama M. Severe congenital ichthyosis of the neonate. Int J Dermatol. 1993;37:722-8. [ Links ] [scielo.br]

"Update on epidemiology, diagnosis, and disease course of atopic dermatitis". Seminars in Cutaneous Medicine and Surgery. https://www.ncbi.nlm.nih.gov/pubmed/27525380 Saunders, SP., Moran, T., Floudas, A., Wurlod, F., Kaszlikowska, A., Irvine, AD. [infantcentre.ie]

Von Hebra first described generalized cutaneous inflammation in 1868 [3].The incidence of erythroderma is high in the Indian subcontinent although epidemiological data on a global scale are lacking. [ncbi.nlm.nih.gov]

Pathophysiology Ichthyosis vulgaris is characterized by onset in early childhood, usually between age 3 and 12 months, with fine scales and varying degrees of dryness of the skin. [emedicine.medscape.com]

Read More Inherited Nonsyndromic Ichthyoses: An Update on Pathophysiology, Diagnosis and Treatment. Am J Clin Dermatol 2018 Feb;19(1):51-66 Department of Medical Sciences, Dermatology, Uppsala University, Uppsala, Sweden. [pubfacts.com]

Pathophysiologic basis for growth failure in children with ichthyosis: an evaluation of cutaneous ultrastructure, epidermal permeability barrier function, and energy expenditure. J Pediatr. 2004;145(1):82–92. 45. Taïeb A, Labrèze C. [dovepress.com]

Further pathophysiological study should be performed to explore this hypothesis. One important additional point that needs to be discussed is the impact of sun avoidance on vitD level in ichthyosis patients. [ojrd.biomedcentral.com]

SUMMARY: The expanding phenotype and genotype of the ichthyoses facilitates accurate clinical diagnosis and permits a deeper knowledge of the epidermal pathophysiology. [expertscape.com]

Bland emollients such as petrolatum-based products should be applied several times a day, especially after bathing, to prevent drying. [link.springer.com]

Topical ointment for preventing infection in preterm infants. Cochrane Database Syst Rev. 2004;(1):CD001150. 47. Gastmeier P, Groneberg K, Weist K, Rüden H. [dovepress.com]

The principles of treatment are moistening the skin, prevention of dehydration, and use of keratolytic agents. [readbyqxmd.com]

Appropriate Prophylaxis and Other Measures to Prevent Readmission. [cancertherapyadvisor.com]

CDC [CN] Centers for Disease Control and Prevention" [CN] DP Publication Date The date that the article was published. 2007/1/31[DP] 2001/3:2001/10[DP] 2009[DP] DTRE Date Revised The date a change was last made to a record as a result of NLM's individual [biomedsearch.com]