Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The structure of the skin and the pattern of alteration in chronic ulcerations associated with deficiency of prolidase have been studied superficially in the past. We examined histologically several biopsies taken from apparently normal skin and from ulcerations afflicting a young woman with such a syndrome. Deposits of amyloid were found within the walls of medium-sized vessels and occasionally occluding their lumens. Impaired cutaneous microcirculation resulting from statis, moderate vasculitis, and abnormal structure of the dermis may be responsible for the regional preponderance of deposits of amyloid and ulcerations in the legs.
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PMID:Pathogenesis of ulcerations in deficiency of prolidase. The role of angiopathy and of deposits of amyloid. 650 15

Two cases of prolidase deficiency in two siblings are presented. The patients complained of the typical clinical symptoms of the disease, including chronic leg ulcerations resembling vasculitis. They were mentally retarded, had typical facial characteristics, splenomegaly, and haematologic anomalies. Biochemical and morphological investigations confirmed the diagnosis. In these cases, alterations of the peripheral nervous system and decreased IgA levels were demonstrated for the first time.
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PMID:Chronic leg ulcerations resembling vasculitis in two siblings with prolidase deficiency. 825 46

Leg ulcers of juvenile onset are uncommon. The infectious origin is fairly frequent but the presence of leg ulcers in children should prompt an investigation into possible underlying causes especially hemangioma, vasculitis, inborn errors of metabolism (i.e. prolidase deficiency), hemoglobinopathies, occult spinal dysraphism and immunodeficiencies. Bacteriological investigations are essential and a skin biopsy specimen may be able to differentiate some of these disorders.
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PMID:[Particular aspects of ulcers in children]. 941 52