Ichthyosis: Symptoms & Treatment
What is ichthyosis
The term ichthyosis derives from ichthy-, the Greek root for “fish.” The fanciful reference is to the skin’s scales, which some consider fish-like. Rather than a single disease, ichthyosis denotes a family of related disorders.
The ichthyoses (plural) are a group of rare skin diseases, whose primary symptoms include dry, thickened and scaly skin. In some forms, the skin blisters and peels, especially in infancy and childhood. Many people with ichthyosis have darkened, rough skin that may appear as scales separated by deep cracks. Mild forms of the disorder, such as mild ichthyosis vulgaris (“vulgaris” is Latin for common), are characterized by dry and/or flaky skin, and may go undiagnosed, or misdiagnosed. Ichthyosis is an inherited, genetic disease which may be present at birth or appear later in life. Ichthyosis is not contagious. Furthermore, these children enjoy touching, hugging, and the same kinds of physical contact that are enjoyed by any other child.
Ichthyosis is a disorder in which the body’s ability to produce the outermost layer of skin, the stratum corneum, is defective. The problem may be visualized as a traffic jam of cells on the skin’s surface. This may result from one or both of two problems. Either the body manufactures the stratum corneum too rapidly (up to 300% of normal), or the stratum corneum fails to shed properly from the surface of the skin. Because the skin is constantly renewing itself, either of these two interruptions in the balance between the making of the skin and its shedding results in the symptoms of ichthyosis: thickened, scaly skin.
Primary symptoms & associated problems
Symptoms can vary significantly from child to child. Some children are affected only locally (for example, only on the hands and feet), while others exhibit total or near-total body involvement. The skin may be reddish, extremely flaky, or the body may be covered with thicker, plate-like scales. Many children have significant scalp involvement, which inhibits hair growth. Some may have hearing problems caused by accumulations of exfoliated skin that binds with natural waxes and blocks (or occludes) the ear canal. Some may have moderate to severe itching. Still others have moderate to severe body odor, which results from unavoidable accumulations of harmless bacteria beneath and between scales. Most children are at an increased risk for skin infection due to breakdowns in the skin’s natural barrier function.
Severely affected children may suffer from fine and/or gross motor impairment due to tautness of skin on the hands, and from tautness and cracking of skin on the soles of the feet. However, the most significant danger for many children with ichthyosis in the classroom, and particularly on the playground, is overheating. Overheating results from the body’s inability to adequately sweat through pores blocked by thickened scale. This is a potentially serious problem, and is detailed more fully in a later section of this booklet.
Disruption of the skin’s barrier function also leads to excessive water loss. This is why the skin becomes so dry. In the extreme, this may lead to dehydration. Children with ichthyosis need to drink plenty of water or juice, and provisions must be made at school to allow this.
One form of ichthyosis deserves special mention: epidermolytic ichthyosis (EI*) (formerly EHK). Children with EI have extremely fragile skin that is prone to blistering or tearing upon relatively mild trauma and is vulnerable to cuts and other skin breaks. Because of this, the risk of infection is higher than normal. Teachers should learn from the child’s parents the best ways to accommodate the special needs of the child with EI.
Following is a summary of some common secondary problems that may affect children with ichthyosis:
- Overheating resulting from active play and/or an overly warm environment
- Body odor resulting from normal accumulation of bacteria between and beneath scale (it is important to note that the child is not unclean and probably bathes more than the average child)
- Hearing Impairment (and in some cases, resulting speech impairment) due to occlusion of the ear canal (some rare forms of ichthyosis involve neurosensory hearing loss)
- Sensitivity to bright sunlight and/or increased susceptibility to eye irritation resulting from ectropion**
- Dry eyes (may need to apply eye drops and/or have difficulty reading long assignments
- Susceptibility to infection due to cracks or breaks in the skin’s barrier
- Difficulty walking, running and/or jumping due to involvement of soles of feet (some children have difficulty wearing shoes)
- Side effects from medications
- Difficulty with gross and/or fine motor skills
- Excessive water loss through the skin, leading to dehydration in extreme situations;
- Possible hair loss, receding hairline, and/or excessive dandruff
- Special nutritional needs due to metabolic demands of excessive skin growth
*EI may also be known as bullous congenital ichthyosiform erythroderma, or simply, BCIE.
**Ectropion is an eversion, or pulling away of the margin or lip of the eyelids, due to the tautness of facial skin.
Because ichthyosis is a life-long condition, and because there is no cure, the goal of treatment is to alleviate symptoms and relieve discomfort. Two kinds of symptoms need attention: primary symptoms (thickened, dry, scaling skin, and in some cases severe blistering), and secondary problems (summarized above).
The goal in treating the primary symptoms of ichthyosis is moisturizing affected areas of skin while promoting exfoliation (normal shedding) of the skin’s outermost layer. Both goals are generally met with a regimen that includes application of topical preparations. Treatment of children with ichthyosis employs three main types of topical preparation: humectants, keratolytics, and emollients.
Humectants are a broad class of topical creams and lotions, which effectively penetrate and moisturize the skin by holding water in the stratum corneum. There are hundreds of effective humectants available over the counter and by prescription, including creams with urea and propylene glycol.
Keratolytics actively promote exfoliation by promoting the dissolution of bonds between individual cells of the stratum corneum, the outermost layer of skin. The most commonly used keratolytics are lactic acid, glycolic acid, and salicylic acid.
Emollients are skin lubricants, usually petrolatum or lanolin based. They moisturize the skin by preventing water from evaporating from the surface.
Children typically self treat their skin or receive a thorough topical treatment regimen from one to four times per day (usually combined with baths), depending on the severity of their condition.
At school, a certain amount of maintenance treatment may be necessary. For this purpose, children with ichthyosis must have on hand their essential treatment needs. Maintenance treatment generally involves periodic applications of topical moisturizing creams or lotions. Accommodations should be in place to allow the child time and privacy to do this. Preschoolers and young children may need a reminder from the teacher to apply lotion after water play, sand play, messy artwork or hand washing. However, most children know they are more comfortable when they use their lotions, and only require the necessary time and a place to do it discretely.
Of utmost importance is avoiding unnecessary self-consciousness and embarrassment. Steps must be taken to provide adequate privacy, and to avoid calling undo attention to the child’s special needs.
Children with ichthyosis are sometimes treated with oral medication. Antibiotics may be prescribed for topical or systemic infections. In cases where a child is subject to chronic itching (pruritus), antihistamines and/or topical medications may be prescribed to help manage the problem. An unfortunate side effect of most antihistamines is that they can make a child drowsy or inattentive. This is usually a temporary effect; if it persists and disrupts the child’s education, a change to an alternate class of antihistamines may be suggested.
Children with EI, particularly, should have a topical antibiotic on hand to apply to incidental cuts and scrapes.
Some severely affected young people may also be treated with systemic retinoids. The retinoids are vitamin A derivatives, are potent drugs, and may have some side effects. Although school personnel will probably not administer treatment, teachers and other school officials should be aware that treatment with retinoids is underway, and should be appraised by parents of the most common side effects.
Children of all ages should be encouraged to be active participants in their own treatment. Taking on the responsibility of self-care will help the child develop a sense of independence, control, and competence, all of which help the child meet the normal developmental tasks of childhood.
Teacher, parent and child
It is very difficult for a child to concentrate on learning when the skin is taut, cracked, sore, or itching. Some symptoms of ichthyosis can make simple activities such as holding a pencil, running and playing, or sometimes even sitting still difficult, if not painful. Students who shed excess skin scales can be taught to brush off shared chairs and tables when moving from one activity to the next.
Because ichthyosis is a complex disorder, the first goal is to ascertain the child’s base level of comfort and physical mobility. The child’s parents are the best source for this information, as well as for updates on the child’s day-to-day care requirements. Parents and teachers might find it helpful to convene a conference prior to beginning a new school or school year. The conference could include parents, school nurse, principal and other appropriate staff such as counselor or physical education instructor. If possible, the child’s physician should also be involved. This will provide a forum for ensuring a well-integrated approach to support the child’s special needs in school. Principals can help by making sure the larger school community is aware of the child’s condition and special needs, so that crossing guards, substitute teachers, and lunchroom workers do not inadvertently contribute to a child’s emotional and physical discomfort. Special Education approaches are considered in a later section of this booklet.
The second goal is easing the child’s transition into the society of his or her classmates. Because ichthyosis is usually quite visible, and may even be considered “disfiguring,” the child’s classmates need to have their natural curiosity satisfied.
This can be handled in any number of ways. Some parents could be available the first day of class to answer questions. The student with ichthyosis probably has a ready response to queries about his or her skin. A teacher could be instrumental in supplying words or phrases to better explain their different appearance. Other common strategies include statements written by parents to teachers that introduce their child as a singer, skater, pianist, etc., who happens to have ichthyosis. Letters may be distributed around the school, and perhaps even to parents of the child’s classmates, particularly in the younger grades. It is critical to consider the age of the children and not to draw undue attention upon the child who looks different. A common question that parents often struggle with is “Do we allow our child to be present during the ichthyosis discussion with the classroom?” There are many benefits for both the child and classmates when they are present during the discussion. The child is able to actively engage in the discussion and hear what is being said regarding their skin disease. It also serves as an opportunity for self-expression that is in a safe and controlled environment.
It is important that classmates understand ichthyosis as best they can. Generally, a straightforward, direct approach is best. The ridicule, isolation and depression so common for children with ichthyosis is only heightened by the ignorance that grows out of evading the issue altogether.
Questions to ask the parent
Of course, a conference is not the only opportunity for a teacher and the child’s parents to interact. It is important that open lines of communication exist on an ongoing basis, and that the teacher and other school officials have the greatest possible access to information that will help raise the quality of the child’s school experience.
The following questions may help the teacher learn important information from the child’s family.
a. Begin with the positive. What are the child’s interests and special skills? What does she enjoy doing? What does she excel at?
b. How extensive is the child’s current medical treatment?
c. What medical treatments must take place during school hours?
d. Are there side effects for these treatments? If so, how can the teacher best help the child cope with them?
e. What considerations need to be made for the child that may be outside the normal scope of classroom or school rules or conventions? For example, does the child need special privileges for moving about the classroom to maintain physical comfort, or the freedom to leave the classroom to drink water when needed? Some children find that sitting on hard surfaces for prolonged periods is painful, and a special cushion may be needed. Clothing is a key issue for children with ichthyosis, and conflicts with dress codes may need to be addressed (including the possible need for brimmed hats or sun glasses out of doors, or the use of hats in general in cases of severe scalp involvement causing embarrassing hair loss or excessive dandruff).
f. What are the child’s and family’s preferred way of handling intrusive questions, or ridicule or abuse from peers and adults?
g. Do changes need to be made in the school environment, particularly regarding excessive heat? Overheating can be a potentially dangerous situation for children with ichthyosis.
h. What considerations and precautions need to be made regarding outdoor play in hot weather? Under what circumstances will the child need sunscreen? Are specially designed cooling vests available for the child? Are teachers and other playground monitors confident in their ability to recognize warning signs of overheating?
i. Do considerations need to be made regarding transportation to and from school?
Once there is a sound understanding of the child’s skills and comfort level, it should be relatively easy to monitor activity and ensure a safe and productive school environment. To repeat the key considerations, make certain the child has ready and discrete access to lotions and medications, and be ever mindful of the child’s difficulty in regulating body temperature.
Overheating: Warning Signs & Remedies
For some children with ichthyosis, the inability of the body to properly regulate its temperature is the most significant and potentially dangerous of secondary effects. The difficulty arises due to the scales and thickened skin, which occlude pores, interrupting the body’s ability to sweat. This can result in a rapid and dangerous rise in the body’s core temperature upon exertion, including normal play. The problem is particularly serious when the air temperature is high, and even more so when high humidity accompanies high temperature. Excessive body temperature can be very dangerous.
In hot weather, or in the gym, the child must have easy access to drinking water. This is essential, and may mean allowing the child to leave the classroom, gym, or playground for water without asking permission or permitting him/her to keep a water bottle with him/her at all times. The child should feel that teachers are advocates in this regard, rather than obstacles to comfort and health.
The best way to avoid dangerous overheating is taking preventive measures and being aware of the early warning signs (see below). Teachers must be prepared to intervene at the first sign of overheating. This may mean ceasing the game or activity which is leading to overheating, removing the child from an overly warm environment, allowing the child to rest and externally cool the child by applying cool, damp wash cloths or spritzing with a bottle of cold water, or take other steps that parents and school officials feel are appropriate. Some children with ichthyosis have therapeutic cooling vests, which they can put on at appropriate times.
It is important to know that the child with ichthyosis will not perspire when overheated. Please note that fans, which cool the body by helping perspiration evaporate, are not effective for a child with ichthyosis. Following are some signs of overheating that teachers and school officials can watch for.
Warning Signs of Overheating
- Reddening of the skin, particularly the face (usually the first early sign, though not necessarily an indicator of serious overheating
- Child may become cranky; skin feels warm/hot to touch, but no sweat is evident; child may feel weak, and may complain of being too warm (teacher should intervene)
- Child becomes increasingly listless or lethargic (this signifies a mid-stage at which active steps must be taken to remedy the situation); body temperature may elevate to 106º F or higher; pulse may be rapid and strong; breathing may be labored
- Child may feel dizzy, faint or pass out and convulsions may ensue (the situation has become critical and aggressive steps must be taken to bring temperature down, including removing child to cool environment, placement in cool bath, if possible, or wrapping in cold, wet towels or sheets; this is a medical emergency and medical assistance must be sought and the parents contacted; if unattended to, coma and death may result.
One mother of a five year old Texan wrote to her son’s school principal and nurse stating, “If the temperature is 85°+, my son will become feisty, irritable, argumentative, then tired and lethargic. Always honor his request for water, shade or air conditioning or he will get heatstroke and die.”
Because a child with ichthyosis may overheat easily, the child may be labeled slow, lazy, or a poor athletic performer by uninformed teachers and classmates. This child is at risk for developing poor self-esteem. The insightful intervention of an understanding coach or teacher can make all the difference in these circumstances.
The true nature of supportive spirit is reflected in the story of a young man with ichthyosis who participated in a twenty-six mile marathon. He was able to complete the race because a group of friends formed a water brigade and doused him at checkpoints along the way to the finish line. Another child won her school’s annual physical education award because, as her mother says, “she never stops trying.” The goal of the caring professional should be to help the child find, set, and respect their own limits and goals.
Like any child, those with ichthyosis are likely to get occasional cuts and scrapes. Unlike other children, however, children with ichthyosis may require uncommon treatments for otherwise common occurrences. Consultation with the child’s parents and, if possible, the physician, should guide treatment for common incidents. Following are a few general guidelines.
- Normal Band-Aids may not work well on children with ichthyosis. Either they fail to adhere well to skin that is lubricated with topical moisturizers, or they may adhere too well and cause significant tearing of the skin when removed. When bandaging is necessary, place a non-adherent sterile dressing over the wound, wrap the entire limb or trunk with a nonstick gauze and use surgical tape to secure the bandaging (i.e., tape on bandage, not on skin)
- If the child in your care has epidermolytic hyperkeratosis (EHK), in which the skin is particularly fragile and blistering is common, consult with parents and the child’s physician for guidelines on treatment and preventive measures
- Due to the same factors, which contribute to the child easily overheating, common fevers due to an otherwise mild illness may also pose a problem. Parents should be contacted if the child develops a fever, which passes a threshold determined in advance by parents, physician and school officials.
- Because childhood illnesses like chicken pox and measles may affect the child with ichthyosis more severely than other children, parents should be informed if there is suspicion of an infectious disease in the student population.
This information is provided as a service to patients and parents of patients who have ichthyosis. It is not intended to supplement appropriate medical care, but instead to complement that care with guidance in practical issues facing patients and parents. Neither the Foundation for Ichthyosis & Related Skin Types, Inc., Publications Review Committee, Medical & Scientific Advisory Board, nor Foundation staff and officials endorse any treatments or products reported in this booklet. All issues pertaining to the care and treatment of patients with ichthyosis should be discussed with a dermatologist experienced in the treatment of this complex disease.
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