General care. Management demands a multidisciplinary approach, with skin barrier preservation as the cornerstone of care. Initial management focuses on use of a humidified, warmed isolette; avoiding frequent and prolonged bathing; minimizing skin punctures; and provision of a supportive surface to minimize friction, pressure, and sheer. Bland emollients are a must. Petrolatum or dimethicone-based products are applied every 4 to 6 hours, especially around breaks and fissures, to maximize skin flexibility and diminish TEWL. After the initial period of membrane drying, deep fissures and peeling can be loosely covered with petroleum-impregnated gauze and Kerlix as a secondary layer.1,2
Colonization with organisms. Many physicians initiate systemic antibiotics if concern for infection exists, but colonization without infection is common. My approach is to avoid toxic percutaneous substances such as silver and betadine. I frequently use dialkyl carbamoyl chloride- (DACC) coated gauze (Cutimed Sorbact; BSN medical Gmbh, Hamburg, Germany) to loosely wrap the affected areas. This product provides an excellent organism binder through hydrophobic interactions, removing undamaged quiescent bacteria without toxic systemic reaction or bacterial byproduct release. The gauze can be wrapped individually around fingers and toes, offering protection from both friction and infection. Occlusive 3% bismuth-impregnated gauze (Xeroform; Cardinal Health, Dublin, OH) is another antimicrobial dressing that provides comfort and lubrication.
Fissures. Deep fissuring often leads to exudate and wound development. PolyMem (Ferris Corp, Fort Worth, TX) is a nonadhesive hydrophilic polyurethane foam/pad that offers a comfortable alternative for these patients. Infused with surfactant cleanser, humectant, and superabsorbers, this dressing can provide absorption and comfort and minimize pain because less cleaning is required and the soft padding is comforting to the skin. Various foams can be an excellent choice for protection, absorption, and padding.
In the neonatal period, medical Leptospermum honey gel/dressing can be helpful in deep fissures, wounds, and mucosal ulcers. Our medical team has observed occasional pain reactions on honey application to the injured skin; these can be addressed by using contact perforated layers such as Mepitel soft silicone dressing (Mölnlycke Health Care, Sweden) to protect denuded skin while allowing topical product applications. As children with ichthyosis get older, nanocrystalline silver-coated foams can be used more often because immature skin and systemic absorption become less of a concern. In addition, skin colonization can be reduced with a diluted bleach bath (safe to do in full-term babies in the neonatal unit) every 3 to 4 days.
Debridement. Minimal debridement is recommended because an aggressive approach can lead to further injury, but significant fissuring and peeling can leave tissue nonviable. Application of a hydrogel or surfactant-based gel makes slough/scale easier to remove. Conservative gentle trimming with scissors tends to work best for me.
For deeper wounds, all of the above management strategies apply; more advanced products such as collagen-based and amniotic membrane-based products also may be utilized.
Corneal drying. Corneal drying can be avoided by applying artificial tears and emollients around the eye area. A 10% urea cream applied to eyelid skin 2 to 3 times per day can prevent skin contracture. Reports of bilayer skin substitute for cicatrical ectropion and amniotic membrane transplant to promote epithelialization have been published.3 Oral membrane care (specifically, fissures on the lips and mouth mucosa) involves use of bland emollients. In my experience, Leptospermum honey gel is helpful for oral mucositis and fissured, dry lips (I often mix it with a bit of petrolatum).
Contractures. Contractures of the hands/feet can lead to ischemia, gangrene, functional deformity, or total loss of digits. Contracture release is important, keeping in mind the painful aspect of this procedure. Gentle physical therapy can be helpful when skin flexibility is greatly diminished, but the clinician must be made aware of the potential for friction-induced injury and pain. We often incorporate a pain management team in the care of these babies.