Ulcus cruris associated with prolidase deficiency.
Prolidase deficiency is an autosomal recessive disorder that is associated with chronic cutaneous ulcers, mental retardation, unusual facial appearance, skeletal deformities, joint dislocations, hematological anomalies, splenomegaly, and chronic infections. The most typical finding is chronic, recurrent leg ulcers appearing in early childhood. Prolidase (peptidase-D) is necessary for collagen biosynthesis and its deficiency leads to impairment in connective tissue of the skin, capillaries, and lymphatic vessels. We report a 33-year-old woman who had a 15-year history of nonhealing ulcer on left pretibial region accompanied by splenomegaly, hypochromic microcytic anemia, and thrombocytopenia. Prolidase deficiency is a rare genodermatosis and must be considered in the differential diagnosis of leg ulcers that develop at an early age.
Kavala
Mukaddes
M
Department of Dermatology, Goztepe Training Hospital, Istanbul, Turkey.
Zindanci
Ilkin
I
Sudogan
Sibel
S
Turkoglu
Zafer
Z
Sarigul
Sukran
S
D000328:Adult; D003094:Collagen / Q000378:metabolism*; D004150:Dipeptidases / Q000172:deficiency*; D017707:Erythrocyte Transfusion; D005260:Female; D006801:Humans; D007871:Leg Ulcer / Q000201:enzymology* / Q000628:therapy; D014945:Wound Healing / Q000502:physiology
D003094:Collagen; D004150:Dipeptidases; C022449:proline dipeptidase