Olmsted Syndrome: Case Report of Nursing Management of Premature Twins
Sara Anderson1, Jacquelyn Paradise, Media Esser
1Children's Wisconsin, Wauwatosa.
Insights
Olmsted syndrome, a rare skin disorder, requires specialized care in premature infants. This case study highlights an evidence-based, family-centered approach for managing extensive skin plaques in fragile newborns.
Area of Science:
- Dermatology
- Neonatology
- Genetics
Background:
- Olmsted syndrome is a rare congenital skin disorder affecting infants, characterized by extensive plaques.
- Premature infants present unique challenges due to their fragile skin, complicating treatment.
- This case study focuses on twin infants born at 28 weeks' gestation with Olmsted syndrome.
Observation:
- Infants presented with numerous skin plaques, necessitating a multidisciplinary diagnostic approach.
- Differential diagnoses included severe palmoplantar keratoderma.
- The primary diagnosis of Olmsted syndrome was confirmed by a specialized team.
Findings:
- A consistent, evidence-based, family-centered care model was implemented for managing skin plaques.
- An interdisciplinary skin care regimen facilitated plaque softening and removal.
- Continuous management is essential to address ongoing plaque buildup.
Implications:
- Highlights the importance of a structured, evidence-based approach for rare dermatological conditions in neonates.
- Emphasizes the need for specialized, consistent care plans for premature infants with complex skin disorders.
- Demonstrates the successful management of Olmsted syndrome through multidisciplinary collaboration and family-centered care.
Background:
Olmsted syndrome is a rare and complex skin disorder affecting 46 (published) infants as of 2012. The infants affected in this case were born premature at 28 weeks' gestation. Infants affected by this syndrome demonstrate numerous plaques on several specific areas of the skin. Common treatments include exfoliation in addition to psoriasis treatments. The extremely fragile nature of the premature infants' skin complicates treatment modalities.
Clinical Findings:
The progression of the infants' dermatologic findings and plaque formation is discussed in this case study.
Primary Diagnosis:
The primary diagnosis of Olmsted syndrome was made with the assistance of a multidisciplinary team to work through several differential diagnoses presenting with severe forms of palmoplantar keratoderma.
Interventions:
The management of skin plaques in twin premature infants is presented in this case study. An evidence-based approach, utilizing the model of family-centered care, is presented with multidisciplinary involvement and an outline of the specific plan of care for the extensive skin care regimen used.
Outcomes:
An interdisciplinary skin care regimen was created to provide consistency in transition from hospital to home. Using a consistent approach, the plaques were able to be softened and many removed. Continual maintenance is required to manage continual buildup of skin plaques.
Practice Recommendations:
Premature infants are at increased risk for infection due to the immaturity of their skin. The complexity of their skin complicates the ability to recognize and care for rare skin disorders. This case study illuminates the practicality of a consistent and evidence-based approach to a complex and rare skin disorder.
