The ABCA12 gene is involved in the highly rare condition known as harlequin ichthyosis (HI), commonly referred to as “ichthyosis fetalis.” It is ichthyosis’s most serious subtype. Mutants of the ABCA12 gene in HI lead to hyperkeratosis, an autosomal-recessive illness that thickens the keratin layer in the stratum corneum of the skin.
Moreover, HI is distinguished by thicker, dry plaques that are “armor-like,” that give the impression of “scales” that are divided by deep fissures and covering the whole surface of the body. Due to the compromised barrier of the skin, infants with this condition are more susceptible to infection.
Epidemiology
1 in 300,000 babies is said to be affected by harlequin ichthyosis. There is no data to suggest male or female susceptibility. No evidence of the disease’s prevalence in terms of racial or sexual distribution has been found.
Anatomy
Pathophysiology
Etiology
As a keratinocyte transmembrane fatty transporter protein, ABCA12 serves this purpose. It is a part of the ATP-binding cassette transporter, which binds and metabolizes ATP to facilitate the transport of phospholipid bilayers across external membranes. The stratum corneum of the epidermis’ extracellular lipid layers, which are formed by lamellar granules, serve as a vehicle for ABCA12’s transportation across extrinsic membranes.
Lipids are improperly transported to the stratum corneum as a consequence of genetic changes in the ABCA12 transport pathway. As a result, hyperkeratosis—an overaccumulation of lipids in the epidermal keratinocytes—occurs when the lipid barrier is damaged. The HI-specific traits are caused by intracellular lipid buildup in the epidermal keratinocytes.
Genetics
Prognostic Factors
High mortality rates are present in HI. According to Rajpopat et al. analysis of 45 cases of HI, there have been 25 survivors & 20 fatalities, for a fatality rate of 44%.
The survivors ranged in age from ten months to twenty – five years. Fulminant sepsis, respiratory failure, or a combination of both were the most frequent causes of mortality in the first three months, accounting for 75% of deaths.
Early administration of oral retinoids may increase survival; 83% of those who received treatment lived, compared to 76% of those who did not.
Clinical History
Clinical History
The clinical history of ichthyosis fetalis may include the following:
Skin abnormalities: The most obvious symptom of ichthyosis fetalis is the presence of a thick, scaly collodion membrane covering the baby’s skin. This membrane may crack and peel, leaving behind red, inflamed skin. The skin may also be abnormally dry and prone to itching.
Respiratory problems: Some infants with ichthyosis fetalis may have respiratory distress due to the thickened skin covering their chest, which can make breathing difficult.
Eye problems: The thickened skin on the eyelids may make it difficult for the baby to open their eyes, and they may be at risk for developing corneal ulcers or infections.
Increased risk of infection: The abnormal skin barrier in ichthyosis fetalis can make the infant more susceptible to infections.
Poor feeding: Infants with ichthyosis fetalis may have difficulty feeding due to the presence of thickened skin on their lips and inside their mouth.
Joint contractures: Some infants with ichthyosis fetalis may develop joint contractures, which are characterized by the inability to fully extend or move certain joints.
Physical Examination
Physical examination
The physical examination of an infant with ichthyosis fetalis typically reveals several distinctive findings related to the skin and other organ systems. Some of the physical examination findings of ichthyosis fetalis may include:
Thick, scaly skin: The hallmark physical finding of ichthyosis fetalis is the presence of a thick, scaly collodion membrane covering the infant’s skin. This membrane may crack and peel, leaving behind red, inflamed skin that is prone to infection.
Facial and body dysmorphism: Infants with ichthyosis fetalis may have characteristic facial and body features, such as a flattened nose, small ears, and a short neck.
Eye abnormalities: The thickened skin on the eyelids may make it difficult for the infant to open their eyes fully, and they may be at risk for developing corneal ulcers or infections.
Respiratory distress: Some infants with ichthyosis fetalis may have difficulty breathing due to the thickened skin on their chest, which can make it difficult for them to expand their lungs fully.
Joint contractures: Infants with ichthyosis fetalis may have limited mobility or range of motion due to joint contractures, which are characterized by the inability to fully extend or move certain joints.
Nail abnormalities: The nails of infants with ichthyosis fetalis may be thickened and discolored, and they may have a characteristic “V” shape at the end.
Poor feeding: Infants with ichthyosis fetalis may have difficulty feeding due to the presence of thickened skin on their lips and inside their mouth.
Other findings: Infants with ichthyosis fetalis may also exhibit signs of systemic illness, such as fever or jaundice.
It is important to note that the physical examination findings of ichthyosis fetalis can vary widely depending on the severity and extent of the condition. A diagnosis of ichthyosis fetalis is typically based on the characteristic skin findings and confirmed with genetic testing.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Differential diagnosis
Ichthyoses are a class of cutaneous diseases characterized by abnormal epidermal differentiation, which is primarily inherited through an autosomal recessive mechanism.
The autosomal recessive congenital ichthyoses (ARCI), which include self-healing collodion babies, lamellar ichthyoses, & congenital ichthyosiform erythroderma, frequently manifest as collodion babies (remove the primary membrane without developing any further skin disease).
Trichothiodystrophy, Sjogren-Larsson Syndrome, & neutral lipid storage syndrome are additional conditions that can accompany “collodion infant.”
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
In order to overcome the challenges involved in caring for neonates affected by HI, newborns must be admitted to a Level III neonatal critical care facility and work as part of an interprofessional team. The team should also include experts in a range of disciplines, such as physical therapy, dermatology, plastic surgery, otolaryngology, neonatology, genetics, occupational therapy, ophthalmology, dietician, & social work, in addition to the ICU physicians & nurses.
ICU management is primarily conciliatory. Neonates should be kept in a humid incubator in a separate room to prevent thermoregulatory problems. Sepsis and bacterial infections are serious risks for newborns. Close attention to vital signs, blood cultures in cases of hemodynamic instability and lethargy, & regular serum electrolyte testing in cases of depletion are all necessary for patients.
Transepidermal water leakage & electrolyte imbalance are caused by skin barrier loss. Clinicians must keep track of daily weight fluctuations, fluid intake, & urine output to prevent dehydration. If purulent drainage is seen, then cultures should be obtained; deep fissures may be in danger of infection. Antibiotics can be used topically for fissures.
Discomfort and pain can result from severe fissuring. NSAIDs, acetaminophen, or opioids should be used to provide adequate pain management. Moreover, because of their reduced flexibility, newborns with HI are more likely to experience feeding difficulties, pneumonia, restricted lung movement, and respiratory failure. These issues include limitations to the anterior chest-wall extension.
To maintain a patent airway, neonates who exhibit symptoms of acute breathing difficulties need to be intubated. Contractures in the hands & feet’s distal extremities are another condition that affects flexibility. Prevent compartment syndrome from epithelial constrictions & ischemia digit necrosis; these require the removal of plaque or a liberal application of moisturizers. Furthermore, ectropion necessitates ophthalmology consultation and raises the risk of exposure to keratitis.
It’s important to lubricate the eyes properly every hour using artificial tears or even other optical lubricants. Eclabium brought on by plaque tension may result in an immobile mouth & insufficient eating. For adequate calorie intake, a nasogastric tube will be needed. To encourage the shedding of stratum corneum, neonates will also need to take one to two daily baths in just water and apply mild emollients on a regular basis such as products made of petroleum.
Due to the possibility of systemic absorption, stay away from strong topical emollients. Due to the brittleness of the skin, self-adherent gauze or wraps should be used instead of tape to secure tubing or lines. The gold standard of treatment in HI includes the use of systemic retinoids together with supportive treatment & liberal usage of bland emollients. Acitretin is a systemic retinoid that accelerates the shedding of hyperkeratotic plates and lessens scaling.
It has a short half-life and a low negative effect profile. With early systemic retinoid administration, mortality & survival are improved. Early use reduces the incidence of digital necrosis and improves digital and thoracic constrictions, which in turn improve breathing & functional movement. A pre-therapeutic evaluation is necessary before starting systemic retinoid treatment, and this is followed by a series of lab tests that include a full blood count, a comprehensive metabolic panel, triglyceride, cholesterol levels, & urine.
The daily dose of acitretin is 0.5 to 1 mg/kg. Based on physical exam evaluations & side effects, acitretin should be adjusted to the lowest effective dose possible. Around six months old, systemic retinoids can be stopped. Local retinoids with 0.1% tazarotene cream are an excellent alternative for treating limb contractures and ectropion if oral medication is not tolerated. Children with HI need long-term care after the newborn stage, as well as regular follow-up care, physical & occupational treatment to maximize the range of motion, & language and speech therapy for kids who exhibit social and cognitive impairment.
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https://www.ncbi.nlm.nih.gov/books/NBK560492/
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The ABCA12 gene is involved in the highly rare condition known as harlequin ichthyosis (HI), commonly referred to as “ichthyosis fetalis.” It is ichthyosis’s most serious subtype. Mutants of the ABCA12 gene in HI lead to hyperkeratosis, an autosomal-recessive illness that thickens the keratin layer in the stratum corneum of the skin.
Moreover, HI is distinguished by thicker, dry plaques that are “armor-like,” that give the impression of “scales” that are divided by deep fissures and covering the whole surface of the body. Due to the compromised barrier of the skin, infants with this condition are more susceptible to infection.
1 in 300,000 babies is said to be affected by harlequin ichthyosis. There is no data to suggest male or female susceptibility. No evidence of the disease’s prevalence in terms of racial or sexual distribution has been found.
As a keratinocyte transmembrane fatty transporter protein, ABCA12 serves this purpose. It is a part of the ATP-binding cassette transporter, which binds and metabolizes ATP to facilitate the transport of phospholipid bilayers across external membranes. The stratum corneum of the epidermis’ extracellular lipid layers, which are formed by lamellar granules, serve as a vehicle for ABCA12’s transportation across extrinsic membranes.
Lipids are improperly transported to the stratum corneum as a consequence of genetic changes in the ABCA12 transport pathway. As a result, hyperkeratosis—an overaccumulation of lipids in the epidermal keratinocytes—occurs when the lipid barrier is damaged. The HI-specific traits are caused by intracellular lipid buildup in the epidermal keratinocytes.
High mortality rates are present in HI. According to Rajpopat et al. analysis of 45 cases of HI, there have been 25 survivors & 20 fatalities, for a fatality rate of 44%.
The survivors ranged in age from ten months to twenty – five years. Fulminant sepsis, respiratory failure, or a combination of both were the most frequent causes of mortality in the first three months, accounting for 75% of deaths.
Early administration of oral retinoids may increase survival; 83% of those who received treatment lived, compared to 76% of those who did not.
Clinical History
The clinical history of ichthyosis fetalis may include the following:
Skin abnormalities: The most obvious symptom of ichthyosis fetalis is the presence of a thick, scaly collodion membrane covering the baby’s skin. This membrane may crack and peel, leaving behind red, inflamed skin. The skin may also be abnormally dry and prone to itching.
Respiratory problems: Some infants with ichthyosis fetalis may have respiratory distress due to the thickened skin covering their chest, which can make breathing difficult.
Eye problems: The thickened skin on the eyelids may make it difficult for the baby to open their eyes, and they may be at risk for developing corneal ulcers or infections.
Increased risk of infection: The abnormal skin barrier in ichthyosis fetalis can make the infant more susceptible to infections.
Poor feeding: Infants with ichthyosis fetalis may have difficulty feeding due to the presence of thickened skin on their lips and inside their mouth.
Joint contractures: Some infants with ichthyosis fetalis may develop joint contractures, which are characterized by the inability to fully extend or move certain joints.
Physical examination
The physical examination of an infant with ichthyosis fetalis typically reveals several distinctive findings related to the skin and other organ systems. Some of the physical examination findings of ichthyosis fetalis may include:
Thick, scaly skin: The hallmark physical finding of ichthyosis fetalis is the presence of a thick, scaly collodion membrane covering the infant’s skin. This membrane may crack and peel, leaving behind red, inflamed skin that is prone to infection.
Facial and body dysmorphism: Infants with ichthyosis fetalis may have characteristic facial and body features, such as a flattened nose, small ears, and a short neck.
Eye abnormalities: The thickened skin on the eyelids may make it difficult for the infant to open their eyes fully, and they may be at risk for developing corneal ulcers or infections.
Respiratory distress: Some infants with ichthyosis fetalis may have difficulty breathing due to the thickened skin on their chest, which can make it difficult for them to expand their lungs fully.
Joint contractures: Infants with ichthyosis fetalis may have limited mobility or range of motion due to joint contractures, which are characterized by the inability to fully extend or move certain joints.
Nail abnormalities: The nails of infants with ichthyosis fetalis may be thickened and discolored, and they may have a characteristic “V” shape at the end.
Poor feeding: Infants with ichthyosis fetalis may have difficulty feeding due to the presence of thickened skin on their lips and inside their mouth.
Other findings: Infants with ichthyosis fetalis may also exhibit signs of systemic illness, such as fever or jaundice.
It is important to note that the physical examination findings of ichthyosis fetalis can vary widely depending on the severity and extent of the condition. A diagnosis of ichthyosis fetalis is typically based on the characteristic skin findings and confirmed with genetic testing.
Differential diagnosis
Ichthyoses are a class of cutaneous diseases characterized by abnormal epidermal differentiation, which is primarily inherited through an autosomal recessive mechanism.
The autosomal recessive congenital ichthyoses (ARCI), which include self-healing collodion babies, lamellar ichthyoses, & congenital ichthyosiform erythroderma, frequently manifest as collodion babies (remove the primary membrane without developing any further skin disease).
Trichothiodystrophy, Sjogren-Larsson Syndrome, & neutral lipid storage syndrome are additional conditions that can accompany “collodion infant.”
In order to overcome the challenges involved in caring for neonates affected by HI, newborns must be admitted to a Level III neonatal critical care facility and work as part of an interprofessional team. The team should also include experts in a range of disciplines, such as physical therapy, dermatology, plastic surgery, otolaryngology, neonatology, genetics, occupational therapy, ophthalmology, dietician, & social work, in addition to the ICU physicians & nurses.
ICU management is primarily conciliatory. Neonates should be kept in a humid incubator in a separate room to prevent thermoregulatory problems. Sepsis and bacterial infections are serious risks for newborns. Close attention to vital signs, blood cultures in cases of hemodynamic instability and lethargy, & regular serum electrolyte testing in cases of depletion are all necessary for patients.
Transepidermal water leakage & electrolyte imbalance are caused by skin barrier loss. Clinicians must keep track of daily weight fluctuations, fluid intake, & urine output to prevent dehydration. If purulent drainage is seen, then cultures should be obtained; deep fissures may be in danger of infection. Antibiotics can be used topically for fissures.
Discomfort and pain can result from severe fissuring. NSAIDs, acetaminophen, or opioids should be used to provide adequate pain management. Moreover, because of their reduced flexibility, newborns with HI are more likely to experience feeding difficulties, pneumonia, restricted lung movement, and respiratory failure. These issues include limitations to the anterior chest-wall extension.
To maintain a patent airway, neonates who exhibit symptoms of acute breathing difficulties need to be intubated. Contractures in the hands & feet’s distal extremities are another condition that affects flexibility. Prevent compartment syndrome from epithelial constrictions & ischemia digit necrosis; these require the removal of plaque or a liberal application of moisturizers. Furthermore, ectropion necessitates ophthalmology consultation and raises the risk of exposure to keratitis.
It’s important to lubricate the eyes properly every hour using artificial tears or even other optical lubricants. Eclabium brought on by plaque tension may result in an immobile mouth & insufficient eating. For adequate calorie intake, a nasogastric tube will be needed. To encourage the shedding of stratum corneum, neonates will also need to take one to two daily baths in just water and apply mild emollients on a regular basis such as products made of petroleum.
Due to the possibility of systemic absorption, stay away from strong topical emollients. Due to the brittleness of the skin, self-adherent gauze or wraps should be used instead of tape to secure tubing or lines. The gold standard of treatment in HI includes the use of systemic retinoids together with supportive treatment & liberal usage of bland emollients. Acitretin is a systemic retinoid that accelerates the shedding of hyperkeratotic plates and lessens scaling.
It has a short half-life and a low negative effect profile. With early systemic retinoid administration, mortality & survival are improved. Early use reduces the incidence of digital necrosis and improves digital and thoracic constrictions, which in turn improve breathing & functional movement. A pre-therapeutic evaluation is necessary before starting systemic retinoid treatment, and this is followed by a series of lab tests that include a full blood count, a comprehensive metabolic panel, triglyceride, cholesterol levels, & urine.
The daily dose of acitretin is 0.5 to 1 mg/kg. Based on physical exam evaluations & side effects, acitretin should be adjusted to the lowest effective dose possible. Around six months old, systemic retinoids can be stopped. Local retinoids with 0.1% tazarotene cream are an excellent alternative for treating limb contractures and ectropion if oral medication is not tolerated. Children with HI need long-term care after the newborn stage, as well as regular follow-up care, physical & occupational treatment to maximize the range of motion, & language and speech therapy for kids who exhibit social and cognitive impairment.
https://www.ncbi.nlm.nih.gov/books/NBK560492/
medtigo
Ichthyosis Fetalis
Updated :
September 5, 2023
The ABCA12 gene is involved in the highly rare condition known as harlequin ichthyosis (HI), commonly referred to as “ichthyosis fetalis.” It is ichthyosis’s most serious subtype. Mutants of the ABCA12 gene in HI lead to hyperkeratosis, an autosomal-recessive illness that thickens the keratin layer in the stratum corneum of the skin.
Moreover, HI is distinguished by thicker, dry plaques that are “armor-like,” that give the impression of “scales” that are divided by deep fissures and covering the whole surface of the body. Due to the compromised barrier of the skin, infants with this condition are more susceptible to infection.
1 in 300,000 babies is said to be affected by harlequin ichthyosis. There is no data to suggest male or female susceptibility. No evidence of the disease’s prevalence in terms of racial or sexual distribution has been found.
As a keratinocyte transmembrane fatty transporter protein, ABCA12 serves this purpose. It is a part of the ATP-binding cassette transporter, which binds and metabolizes ATP to facilitate the transport of phospholipid bilayers across external membranes. The stratum corneum of the epidermis’ extracellular lipid layers, which are formed by lamellar granules, serve as a vehicle for ABCA12’s transportation across extrinsic membranes.
Lipids are improperly transported to the stratum corneum as a consequence of genetic changes in the ABCA12 transport pathway. As a result, hyperkeratosis—an overaccumulation of lipids in the epidermal keratinocytes—occurs when the lipid barrier is damaged. The HI-specific traits are caused by intracellular lipid buildup in the epidermal keratinocytes.
High mortality rates are present in HI. According to Rajpopat et al. analysis of 45 cases of HI, there have been 25 survivors & 20 fatalities, for a fatality rate of 44%.
The survivors ranged in age from ten months to twenty – five years. Fulminant sepsis, respiratory failure, or a combination of both were the most frequent causes of mortality in the first three months, accounting for 75% of deaths.
Early administration of oral retinoids may increase survival; 83% of those who received treatment lived, compared to 76% of those who did not.
Clinical History
The clinical history of ichthyosis fetalis may include the following:
Skin abnormalities: The most obvious symptom of ichthyosis fetalis is the presence of a thick, scaly collodion membrane covering the baby’s skin. This membrane may crack and peel, leaving behind red, inflamed skin. The skin may also be abnormally dry and prone to itching.
Respiratory problems: Some infants with ichthyosis fetalis may have respiratory distress due to the thickened skin covering their chest, which can make breathing difficult.
Eye problems: The thickened skin on the eyelids may make it difficult for the baby to open their eyes, and they may be at risk for developing corneal ulcers or infections.
Increased risk of infection: The abnormal skin barrier in ichthyosis fetalis can make the infant more susceptible to infections.
Poor feeding: Infants with ichthyosis fetalis may have difficulty feeding due to the presence of thickened skin on their lips and inside their mouth.
Joint contractures: Some infants with ichthyosis fetalis may develop joint contractures, which are characterized by the inability to fully extend or move certain joints.
Physical examination
The physical examination of an infant with ichthyosis fetalis typically reveals several distinctive findings related to the skin and other organ systems. Some of the physical examination findings of ichthyosis fetalis may include:
Thick, scaly skin: The hallmark physical finding of ichthyosis fetalis is the presence of a thick, scaly collodion membrane covering the infant’s skin. This membrane may crack and peel, leaving behind red, inflamed skin that is prone to infection.
Facial and body dysmorphism: Infants with ichthyosis fetalis may have characteristic facial and body features, such as a flattened nose, small ears, and a short neck.
Eye abnormalities: The thickened skin on the eyelids may make it difficult for the infant to open their eyes fully, and they may be at risk for developing corneal ulcers or infections.
Respiratory distress: Some infants with ichthyosis fetalis may have difficulty breathing due to the thickened skin on their chest, which can make it difficult for them to expand their lungs fully.
Joint contractures: Infants with ichthyosis fetalis may have limited mobility or range of motion due to joint contractures, which are characterized by the inability to fully extend or move certain joints.
Nail abnormalities: The nails of infants with ichthyosis fetalis may be thickened and discolored, and they may have a characteristic “V” shape at the end.
Poor feeding: Infants with ichthyosis fetalis may have difficulty feeding due to the presence of thickened skin on their lips and inside their mouth.
Other findings: Infants with ichthyosis fetalis may also exhibit signs of systemic illness, such as fever or jaundice.
It is important to note that the physical examination findings of ichthyosis fetalis can vary widely depending on the severity and extent of the condition. A diagnosis of ichthyosis fetalis is typically based on the characteristic skin findings and confirmed with genetic testing.
Differential diagnosis
Ichthyoses are a class of cutaneous diseases characterized by abnormal epidermal differentiation, which is primarily inherited through an autosomal recessive mechanism.
The autosomal recessive congenital ichthyoses (ARCI), which include self-healing collodion babies, lamellar ichthyoses, & congenital ichthyosiform erythroderma, frequently manifest as collodion babies (remove the primary membrane without developing any further skin disease).
Trichothiodystrophy, Sjogren-Larsson Syndrome, & neutral lipid storage syndrome are additional conditions that can accompany “collodion infant.”
In order to overcome the challenges involved in caring for neonates affected by HI, newborns must be admitted to a Level III neonatal critical care facility and work as part of an interprofessional team. The team should also include experts in a range of disciplines, such as physical therapy, dermatology, plastic surgery, otolaryngology, neonatology, genetics, occupational therapy, ophthalmology, dietician, & social work, in addition to the ICU physicians & nurses.
ICU management is primarily conciliatory. Neonates should be kept in a humid incubator in a separate room to prevent thermoregulatory problems. Sepsis and bacterial infections are serious risks for newborns. Close attention to vital signs, blood cultures in cases of hemodynamic instability and lethargy, & regular serum electrolyte testing in cases of depletion are all necessary for patients.
Transepidermal water leakage & electrolyte imbalance are caused by skin barrier loss. Clinicians must keep track of daily weight fluctuations, fluid intake, & urine output to prevent dehydration. If purulent drainage is seen, then cultures should be obtained; deep fissures may be in danger of infection. Antibiotics can be used topically for fissures.
Discomfort and pain can result from severe fissuring. NSAIDs, acetaminophen, or opioids should be used to provide adequate pain management. Moreover, because of their reduced flexibility, newborns with HI are more likely to experience feeding difficulties, pneumonia, restricted lung movement, and respiratory failure. These issues include limitations to the anterior chest-wall extension.
To maintain a patent airway, neonates who exhibit symptoms of acute breathing difficulties need to be intubated. Contractures in the hands & feet’s distal extremities are another condition that affects flexibility. Prevent compartment syndrome from epithelial constrictions & ischemia digit necrosis; these require the removal of plaque or a liberal application of moisturizers. Furthermore, ectropion necessitates ophthalmology consultation and raises the risk of exposure to keratitis.
It’s important to lubricate the eyes properly every hour using artificial tears or even other optical lubricants. Eclabium brought on by plaque tension may result in an immobile mouth & insufficient eating. For adequate calorie intake, a nasogastric tube will be needed. To encourage the shedding of stratum corneum, neonates will also need to take one to two daily baths in just water and apply mild emollients on a regular basis such as products made of petroleum.
Due to the possibility of systemic absorption, stay away from strong topical emollients. Due to the brittleness of the skin, self-adherent gauze or wraps should be used instead of tape to secure tubing or lines. The gold standard of treatment in HI includes the use of systemic retinoids together with supportive treatment & liberal usage of bland emollients. Acitretin is a systemic retinoid that accelerates the shedding of hyperkeratotic plates and lessens scaling.
It has a short half-life and a low negative effect profile. With early systemic retinoid administration, mortality & survival are improved. Early use reduces the incidence of digital necrosis and improves digital and thoracic constrictions, which in turn improve breathing & functional movement. A pre-therapeutic evaluation is necessary before starting systemic retinoid treatment, and this is followed by a series of lab tests that include a full blood count, a comprehensive metabolic panel, triglyceride, cholesterol levels, & urine.
The daily dose of acitretin is 0.5 to 1 mg/kg. Based on physical exam evaluations & side effects, acitretin should be adjusted to the lowest effective dose possible. Around six months old, systemic retinoids can be stopped. Local retinoids with 0.1% tazarotene cream are an excellent alternative for treating limb contractures and ectropion if oral medication is not tolerated. Children with HI need long-term care after the newborn stage, as well as regular follow-up care, physical & occupational treatment to maximize the range of motion, & language and speech therapy for kids who exhibit social and cognitive impairment.
https://www.ncbi.nlm.nih.gov/books/NBK560492/
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