HistoryThis section has been translated automatically.
Goodman, 1968
DefinitionThis section has been translated automatically.
Rare, hereditary disorder of the enzyme prolidase and the resulting disturbances in collagen degradation.
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EtiopathogenesisThis section has been translated automatically.
Controversial. It is assumed that autosomal recessive heterogeneous mutations in the prolidase gene at the gene locus 19q12-q13.2 are inherited.
ManifestationThis section has been translated automatically.
Mostly occurring in childhood or puberty.
LocalizationThis section has been translated automatically.
Lower extremity.
Clinical featuresThis section has been translated automatically.
Initially mostly solitary, disseminated, reddened papules or small erosions. In the further course of the disease increasing, bizarrely limited, smeary-coated ulcerations with hyperpigmented surroundings, which are almost always resistant to therapy and very painful. S.a. ulcus cruris. Mostly complex accompanying symptoms, including purpura, poikiloderma, wound healing disorders, increased sensitivity to light, canities, keratosis follicularis, intelligence disorders.
LaboratoryThis section has been translated automatically.
The prolidase activity in erythrocytes, fibroblasts and plasma is reduced. Hypochromic anemia and hypergammaglobulinemia often exist. Urine screening: Content of proline or hydroxyproline containing dipeptides (electrophoresis/chromatography) is increased.
DiagnosisThis section has been translated automatically.
Clinic, laboratory, Doppler sonography of the leg arteries, histology.
TherapyThis section has been translated automatically.
Internal and external symptomatic therapy. Trial with vitamin C (e.g. Cebion N) 400-1000 mg/day p.o. Temporarily successful in studies: apheresis of erythrocytes.
LiteratureThis section has been translated automatically.
- Bamberger N (1991) Leg ulcers of juvenile onset. Lancet 337: 1172-1173
- Dyne K et al (2001) Mild, late-onset prolidase deficiency: another Italian case. Br J Dermatol 144: 635-636
- Goodman SI et al (1968) A syndrome resembling lathyrism associated with imiodipeptiduria. On J Med 45: 152-159
- Box R et al (2000) Hereditary prolidase deficiency. Contribution to differential therapy refractory leg ulcer diagnosis. dermatologist 51: 846-851
- Kokturk A et al (2002) Prolidase deficiency. Int J Dermatol 41: 45-48
- Lupi A et al (2002) Therapeutic apheresis exchange in two patients with prolidase deficiency. Br J Dermatology 147: 1237-1241
- Milligan A et al (1989) Prolidase deficiency: a case report and literature review. Br J Dermatol 121: 405-409
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Incoming links (1)
PID;Outgoing links (5)
Canities (overview); Keratosis pilaris; Leg ulcer; Poikiloderma (overview); Purpura (overview);Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer