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Complications due to prematurity
| Provide moist environment in highly humidified incubator (50%-70% humidity). Prevent infection using hygienic standard precautions. Perform regular bacteriologic samplings to monitor for skin & systemic infections. Monitor & maintain body temperature to avoid hypothermia or overheating. Monitor & maintain weight & water & electrolyte balance to avoid hypernatremia or dehydration. Manage pain as needed.
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Pulmonary
| If pulmonary ventilation is suppressed due to skin constriction or nasal occlusion, endotracheal intubation may be required. | Mostly limited to infants w/severe HI |
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Vascular manifestations in extremities
| In neonates & infants: massaging of constriction bands w/emollients may soften skin & improve vascular circulation; if ischemia is evident, topical retinoids or surgical linear band incisions may be considered. | |
| In older children & adults: physical therapy for contractures, arthritis, pain |
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Dermatologic (topical therapy)
| In neonates & infants: frequent daily application of bland emollients, such as petrolatum-based creams & ointments to keep skin soft, supple, & hydrated | |
| In older children & adults: humidification w/long baths, lubrication & keratolytic agents (e.g., alpha-hydroxy acid, urea preparations; vitamin D3 derivatives) to promote peeling & thinning of stratum corneum | In young children, topical salicylic acid preparations should be avoided because of absorption through skin leading to toxicity. Topical use of tacrolimus or vitamin D should be considered w/caution due to skin absorption. Skin irritation may limit use of keratolytics.
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| Topical retinoids (tazarotene, tretinoin, & adapalene) to ↓ scaling, reverse ectropion, & digital contractures | Systemic laboratory monitoring is not recommended for topical use. |
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Dermatologic (systemic therapy)
| In neonates: treatment w/systemic retinoids usually unnecessary, but may be considered in neonates w/severe HI | For long-term use of systemic retinoids, important safety & health concerns must be considered. Treatment requires careful risk vs benefit assessment & monitoring, & should be performed by dermatologist familiar w/ congenital ichthyosis. Oral retinoid therapy should be used w/caution in women of child-bearing age because of teratogenicity (see Pregnancy Management). Additional side effects of retinoids incl hypertriglyceridemia, hepatotoxicity, bone toxicity & ligamentous calcifications, dry eyes, night blindness, & retinal dysfunction from long-term use. For a detailed review of choice of retinoid, dosage, treatment duration, toxicity, monitoring, & disease-specific considerations for ARCI, see Zaenglein et al [2021].
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In children & adults:
Moderate to severe ARCI often necessitates systemic (oral) retinoid therapy. Oral retinoids (isotretinoin, etretinate) can effectively ↓ hyperkeratosis & scaling but are less beneficial in suppressing erythroderma.
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Temperature stability
| Frequent fluid intake & moistening of skin w/water (e.g., spray bottle) or use of cooling vests, air conditioning, & humidifiers may prevent overheating & heat intolerance. | Avoidance of extreme temperatures |
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Fluids/Nutrition
| In neonates:
Monitor weight daily as indicator for sufficient fluid & nutrient intake. High-calorie diet; nutritional support via oro- or nasogastric tube may be necessary due to high caloric demand, &/or complications from eclabium. Prevent dehydration.
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| In children & adults: ↑ intake of high-calorie foods, fluids, & protein is necessary, esp for persons w/severe erythroderma. | |
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Infectious disease
| Antiseptics, antifungal topicals in macerated skin areas Prompt treatment of skin or systemic bacterial infection according to antibiotic sensitivity profile
| Prophylactic antibiotic treatment may be considered for neonates w/HI. |
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Ophthalmologic
| Lubrication of cornea w/artificial tears or prescription ophthalmic ointments, esp at night, is helpful in preventing desiccation of cornea. | In persons w/ectropion |
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ENT
| Regular removal of built-up scale in external ear canals; regular hearing eval | |
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Skin cancer
| Standard treatments for skin cancers | |
Family support
& resources
| Patient advocacy groups to provide family support & other resources Disability resources & information Financial aid programs Children's camps (Camp Discovery, Camp Wonder)
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