6533b81ffe1ef96bd1277384
RESEARCH PRODUCT
Sequential and combined treatment of prolidase deficiency leg ulcers
I CuppariV MattalianoG MazzolaS SerantoniF P PalumboBianca CudiaG Dianasubject
medicine.medical_specialtyPathologyProlidase deficiencybusiness.industryGeriatrics gerontologyPatient affectedCutaneous lesionUrinemedicine.diseaseGastroenterologyHydroxyprolinechemistry.chemical_compoundSettore MED/18 - Chirurgia GeneraleSkin fragilityCombined treatmentchemistryInternal medicineprolidase deficiencyleg ulcerMedicineGeriatrics and GerontologyMeeting abstractbusinessplatelet geldescription
Background The Authors report a case of chronic cutaneous lesions in a patient affected by prolidase deficiency, a rare disorder inherited through an autosomal recessive gene (50 cases reported ). The enzyme prolidase is widely distributed throughout the body and it is important in the recycling of proline and hydroxyproline. Among the clinical presentations, the most striking manifestation is the skin fragility with leg ulceration (see Table Table11) Table 1 Clinical signs of prolidase deficiency The deficiency of the enzyme prolidase is responsible for massive loss of proline in the urine which is estimated to be as high as 3 g/die. The diagnosis is ascertained by iminopeptiduria greater than 5 mmol/24h. A characteristic feature is absolute resistance to all forms of treatment including rejection of skin grafts.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2010-05-01 | BMC Geriatrics |