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Generalized epidermolytic ichthyosis with palmoplantar hyperkeratosis
Prasta Bayu Putra, Sunardi Radiono, Retno Danarti1
1Department of Dermatology and Venereology, Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada/Dr Sardjito Hospital, Yogyakarta. danarti@ugm.ac.id.
Dermatology Online Journal
|April 5, 2021
Summary
Epidermolytic ichthyosis (EI) is a rare genetic skin disorder. This case study details a 2-year-old girl diagnosed with generalized EI, showing improvement with topical treatments.
Area of Science:
- Dermatology
- Genetics
- Histopathology
Background:
- Epidermolytic ichthyosis (EI) is a rare autosomal dominant keratinization disorder.
- Mutations in keratin 1 or 10 genes cause EI.
- Clinical presentation varies based on palmoplantar hyperkeratosis and skin involvement extent.
Observation:
- A 2-year-old girl presented with generalized erythematous, thick scaling skin and erosions since birth.
- Initial presentation included flaccid blisters that readily formed erosions.
- Histopathology revealed diffuse parakeratosis with vacuolar and granular degeneration in the epidermis.
Findings:
- The patient was diagnosed with generalized EI with palmoplantar hyperkeratosis based on clinical and histopathological findings.
- Genetic testing can confirm the diagnosis.
- Treatment involved mupirocin cream, sodium bicarbonate baths, and moisturizers.
Implications:
- Early diagnosis and management of EI are crucial for improving patient outcomes.
- Topical treatments can lead to clinical improvement in EI patients.
- Understanding the clinical and histopathological features aids in diagnosing this rare disorder.
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