[Prolidase and manganese deficiency. Apropos of a case: diagnosis and treatment].
Prolidase deficiency, transmitted on an autosomic recessive mode upsets skin healing and facilitates the occurrence of chronic cutaneous ulcerations. A 36-year-old woman has been followed since the age of 12 for ulcerations and erythematous erysipelatoid plaques of the lower limbs. Two episodes of agranulocytosis were induced by intake of sulfonamides at the age of 17. The same accident had been observed in her aunt. As the aetiological research of ulcers was negative, a prolidase deficit was suspected. The diagnosis is ascertained by the existence of an immunopeptiduria of 5 mmol/24 hours (normally absent). The predominating dipeptides are glycilproline and phenylalanine proline. R-hydroxyproline dipeptide were present at a lesser degree. Urinary hydrolysis showed elevation of free proline (x 10) and hydroxyproline (x 6). Dosage of erythrocytes prolidase evidenced an activity 2 p. 100 of the normal in one case and 55 p. 100 and 49 p. 100 in the parents. Treatment by cofactors of prolidase (vitamine C and manganese) reduced immunopeptiduria, suppressed inflammatory outbreaks and allowed a transient cicatrisation. This tenth case of prolidase deficiency underlines the character of the disease: recurrent ulcers (7/10), erysipelatoid plaques (3/10), ecchymosis (4/10), telangiectatic scars (7/10), edema (1/10), early canitias (1/10). Partial correction by cofactors evokes a prolidase deficiency by inactivation of the enzymes activating systems.
D000328:Adult; D000596:Amino Acids / Q000652:urine; D004150:Dipeptidases / Q000097:blood / Q000172:deficiency*; D005260:Female; D006801:Humans; D007871:Leg Ulcer / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D008345:Manganese / Q000172:deficiency*; D008661:Metabolism, Inborn Errors / Q000175:diagnosis*
D000596:Amino Acids; D008345:Manganese; D004150:Dipeptidases; C022454:prolyl dipeptidase; C022449:proline dipeptidase
Déficit en prolidase et en manganèse. A propos d'une observation : diagnostic et traitement.