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ichthyosis

Collodion Baby

Teaser: 

Dylan Hollman,1Ou Jia (Emilie) Wang,2 Joseph M. Lam, MD, FRCPC,3

1Faculty of Medicine, University of Alberta.2 Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
3Department of Pediatrics, Department of Dermatology and Skin Sciences, University of British Columbia, Vancouver, British Columbia, Canada.

CLINICAL TOOLS

Abstract: Collodion baby, estimated to occur in 1 in 100,000 newborns, is a visually striking clinical presentation seen in neonates that is often a sign of an underlying autosomal recessive congenital ichthyosis. The baby is wrapped in a taut, translucent membrane, which is often compared to plastic wrap, saran wrap, a cocoon, or armour. A formal clinical diagnosis is often not reached until shedding of the membrane reveals the underlying phenotype. This can be isolated or associated with other structural and systemic congenital abnormalities. Patients may require ongoing monitoring and sometimes surgical intervention. Collodion baby is a rare and challenging condition that requires multimodal management including dermatologic care, infection prevention, nutritional support, developmental monitoring, and procedural interventions, if needed.
Key Words: Collodion baby, ichthyosis, neonate, newborn, pediatrics, dermatology.

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Collodion baby is both a diagnosis and a clinical manifestation in newborns who commonly have autosomal recessive congenital ichthyosis.
A highly compromised skin barrier puts the patient at a high risk of both hypo-/hyperthermia, dehydration, poor growth, infection and several other organ-specific complications. Due to these increased risks, admission to the neonatal intensive care unit is necessary to facilitate close monitoring and access to a highly humidified incubator.
The collodion membrane (CM) is shed within 3 to 4 weeks, revealing the underlying ichthyosis. Special investigations can be undertaken before the membrane sheds such as a skin biopsy or blood work. These investigations can provide clinical clues to an earlier diagnosis. If the patient is stable, it is reasonable to wait for the membrane shedding to reveal an underlying diagnosis.
Petroleum-based moisturizers can protect the skin as the membrane peels off.
The most common underlying diagnoses of collodion baby are congenital ichthyosiform erythroderma and lamellar ichthyosis. However, an estimated 10% of patients will have near normal-appearing skin, referred to as self-improving collodion ichthyosis.
Skin barrier dysfunction can lead to significantly higher transepidermal water loss and poor temperature regulation. A highly humidified incubator (minimum 60%) can help reduce water loss and assist in adequate temperature regulation.
Other keys to management include close observation for signs of infection, dehydration, electrolyte imbalance and/or poor feeding/decreased growth velocity.
Topical petroleum-based lubricants should be applied multiple times per day while medicated ointments should be avoided due to risk of systemic toxicity.
Complications involving the lungs (chest constriction or respiratory distress), eyes (ectropion or keratitis) and ears (obstruction, conductive and sensorineural hearing loss) may also be seen. In these instances, consultations with pulmonology, ophthalmology or otorhinolaryngology may be necessary for comprehensive care.
Skin biopsy prior to membrane shedding is generally unhelpful but may provide disease-specific histological findings if done after the collodion sheds.
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Xerosis and Ichthyosis: A Brief Review

Teaser: 

Manish Toofany,1 Joseph M. Lam, MD, FRCPC,2

1 Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
2Department of Pediatrics, Department of Dermatology and Skin Sciences, University of British Columbia, Vancouver, British Columbia, Canada.

CLINICAL TOOLS

Abstract: Xerosis is a common skin condition that becomes more prevalent with age and is a prominent feature in ichthyosis. It primarily resulting from abnormalities in the stratum corneum, influenced by factors like natural moisturizing factors, lipid synthesis, and genetic pathologic variants. The diagnosis of xerosis and ichthyosis is usually made clinically, though specific investigations may aid in the diagnosis of specific ichthyoses. Both conditions can have a considerable negative impact on the quality of life of patients. Management includes moisturization and taking preventive measures to maintain skin health and to prevent xerosis.
Key Words: xerosis, ichthyosis, stratum corneum, natural moisturizing factors.

Members of the College of Family Physicians of Canada may claim MAINPRO-M2 Credits for this unaccredited educational program.

www.cfpc.ca/Mainpro_M2

You can take quizzes without subscribing; however, your results will not be stored. Subscribers will have access to their quiz results for future reference.

Xerosis is a common condition worldwide and it is a prominent feature of both syndromic and non-syndromic ichthyoses.
Both xerosis and ichthyosis can have a considerable negative impact on the quality of life of patients.
The diagnosis of xerosis and inherited ichthyoses is based on history, clinical manifestations, associated abnormalities, and family history, but several specific investigations may aid in the diagnosis of syndromic ichthyoses.
Managing xerosis includes applying topical moisturizers with key components like lipids and humectants while proactive preventive measures are vital for preventing triggers of the condition.
Understanding and addressing triggers such as temperature, low humidity, sunlight exposure, and environmental conditions like air conditioning or heating can be essential in managing xerosis effectively.
Evaluating the overall severity of xerosis and ichthyosis may include the use of established tools like the Dermatology Life Quality Index (DLQI) and the newer Ichthyosis Scoring System (ISS) to assess their impact on patients’ quality of life.
Identifying signs such as brittle hair, neurological abnormalities, and palmoplantar keratoderma can be suggestive of syndromic ichthyoses. These indicators are valuable for recognizing these rare conditions and guiding further examination or evaluation.
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Disclaimer: 
Disclaimer at the end of each page