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Sepsis as an unusual event in dyskeratosis follicularis
V Milavec-Puretić1, J Lipozencić, N Sustić
1Department of Dermatovenerology, Zagreb University Hospital Center, Salata 4, 10000 Zagreb, Croatia. nina@mef.hr
Abstract:
Dyskeratosis follicularis is a genetic disorder characterized by pathogenetic changes of keratinization. We report on a severe case of the disease with an unusual manifestation involving Staphylococcal sepsis. The patient was treated systemically with infusions, oral antibiotics, and retinoids. Antiseptics, keratolytic ointments, and creams were given topically to promote epithelization. His condition improved dramatically after 14 days of treatment. All erosions of the trunk, extremities, neck, and head had epithelized. We suspect that extreme sun exposure and neglect of care on genetically susceptible sites triggered the sepsis.
Insights
Dyskeratosis follicularis, a genetic skin disorder, can lead to severe Staphylococcal sepsis. Prompt treatment with systemic and topical therapies resulted in dramatic epithelization and recovery.
Area of Science:
- Dermatology
- Genetics
- Infectious Diseases
Background:
- Dyskeratosis follicularis (Darier disease) is a rare genodermatosis.
- It involves abnormal keratinization and can predispose to infections.
Observation:
- A severe case of Dyskeratosis follicularis presented with Staphylococcal sepsis.
- The patient experienced extensive skin erosions on the trunk, extremities, neck, and head.
Findings:
- Systemic treatment included infusions, oral antibiotics, and retinoids.
- Topical therapy involved antiseptics, keratolytic ointments, and creams to promote healing.
- The patient showed dramatic improvement with complete epithelization within 14 days.
Implications:
- This case highlights a severe, unusual manifestation of Dyskeratosis follicularis.
- Environmental factors like sun exposure and poor hygiene may trigger sepsis in susceptible individuals.
- Effective management requires a combination of systemic and topical treatments.