Health Condition 1: Q812- Epidermolysis bullosa dystrophica
Conditions
Interventions
Intervention1: Losartan: Dose to be administered: 25-100 mg/ day
Dosage per kg body weight: 0.7-1 mg/kg day (not to exceed 1.4 mg/kg/day or 100 mg whichever is lower) in both pediatric and adult patie
Sponsors
Dr Divya Gupta
Eligibility
Inclusion criteria
Inclusion criteria: Clinical: patients with clinical features of DEB- skin fragility, spontaneous onset blistering since birth, scarring, milia, systemic involvement Age > 5 year Written informed consent from patient/ parents/ legal guardian AND Investigational: diagnosis confirmed by immunofluorescence Ag mapping (IFM) AND clinical exome sequencing
Exclusion criteria
Exclusion criteria: Anemia with Hb Hypotension (defined as age-related systolic BP less than 5th percentile) Cardiologic contra-indications- ejection fraction Severe electrolyte imbalances (esp raised serum potassium) Renal artery stenosis Renal or liver impairment Hypersensitivity to losartan or any of the excipients Pregnancy and lactation Currently on drugs that can interact with losartan (e.g. ACE-inhibitors, rifampicin etc)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Number of wounds, Body surface involvement (BSA) Pain measurement on Visual Analogue Scale (VAS) Pruritus measurement on Visual Analogue Scale (VAS) Physician Global Assessment (PGA) Decrease in the usage of dressings Birmingham Epidermolysis Bullosa Severity Score (BEBS) Epidermolysis bullosa Disease Activity and Scarring Index (EBDASI) instrument for scoring clinical outcome for research in EB- (iscor-EB) Timepoint: 0,3,6 and 9 months | — |
Secondary
| Measure | Time frame |
|---|---|
| Levels of IL-6, TGF-β, Smad2, Smad 6 in the woundsTimepoint: 0 and 9 months | — |
Countries
India
Contacts
Public ContactDr Divya Gupta
Division of Pediatric Dermatology, Manipal Hospital, Bengaluru
Outcome results
None listed