Collodion baby Q80.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Definition
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Clinically descriptive term for a perinatal condition that can occur in various forms of ichthyosis. No independent clinical picture. In most cases an autosomal recessive hereditary lamellar ichthyosis (ARCI) is present. However, collodion membranes are also observed in other forms of ichthyosis.

Manifestation
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From birth.

Clinical features
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Diffuse redness, lamellar scaling, sometimes brownish, creased, dry, parchment-like skin coating over the entire body. Mostly presence of an ectropion. Combinations with hypotrichosis, nail changes, heart defects, short stature, oligophrenia, microophthalmia, deep corneal clouding or epilepsy are also possible. Skin detachment within a few days to weeks. The further course is different. Often a lamellar ichthyosis (see below Ichthyosis) develops, in some cases the skin completely normalizes within weeks to months. Collodion babies are newborns at risk and require special monitoring.

General therapy
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In cooperation with the pediatricians symptomatic therapy to survive the process of skin detachment without complications. Usually the skin detaches within the first 3 weeks. Manual tuition should be avoided. Important are storage in a moist incubator (increased water and heat loss), balancing of water and electrolyte balance (hypernatremic dehydration), avoidance of secondary infections as well as intensive medical monitoring. If ectropion is present, ophthalmological consultation and protection of the eyes from dehydration and external injury are required.

External therapy
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In case of tears in the skin fat gauze with antiseptic additives such as chlorhexidine (e.g. Bactigras gauze dressing). After removal of the skin, re-fattening external agents such as Linola Cream, Ash Base Cream, Base Cream (DAC), if necessary for light keratolysis with additions of 2-5% urea R102 or vitamin A acid R256.

Internal therapy
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Depending on the degree of severity, prophylactic or, in case of superinfection, systemic antibiosis with cephalosporins, as soon as possible after antibiogram.

Progression/forecast
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Different. Depending on the classification of the underlying form of ichthyosis. Approximately 10% of children with collodion skin or congenital ichthyosiform erythroderma experience almost complete healing of the ichthyosis within the first months of life. This is caused by a pressure-sensitive mutation in the transglutaminase 1 gene.

Literature
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  1. Akcakus M et al (2003) Collodion baby associated with asymmetric crying facies: a case report. Pediatric dermatol 20: 134-136
  2. DiGiovanna JJ et al (2003) Ichthyosis: etiology, diagnosis, and management. At J Clin Dermatol 4: 81-95
  3. Ganemo A et al (2003) Autosomal recessive congenital ichthyosis in Sweden and Estonia: clinical, genetic and ultrastructural findings in eighty-three patients. Acta Derm Venereol 83: 24-30
  4. Langer K et al (1991) Collodion baby with transition to mild ichthyosis. Dermatologist 42: 34-38
  5. Taieb A et al (2002) Collodion baby: what's new. J Eur Acad Dermatol Venereol 16: 436-437
  6. Van Gysel D et al (2002) Collodion baby: a follow-up study of 17 cases. J Eur Acad Dermatol Venereol 16: 472-475

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020