Acquired cutis laxa concomitant with nephrotic syndrome.
A 41-year-old woman developed laxity and wrinkling of the skin. This process spread slowly to involve the skin of almost the entire body, without any previous inflammatory skin disorder. The skin of her face, neck, and trunk hung in loose folds, leading to a prematurely aged appearance. One year later she noticed edema on her face and legs. Laboratory studies disclosed low C3 and CH50 serum levels and proteinuria. Skin biopsy specimens revealed extensive loss and fragmentation of dermal elastic fibers. A renal biopsy specimen showed membranoproliferative glomerulonephritis (type 2), and an immunofluorescent study disclosed C3 and IgG deposition in the mesangial matrix and along the glomerular basement membrane. We propose that, in this case, cutis laxa may be related to an abnormal immune response.
Tsuji
T
T
Imajo
Y
Y
Sawabe
M
M
Kuniyuki
S
S
Ishii
M
M
Hamada
T
T
Ishimura
E
E
Hamada
N
N
Nishisawa
Y
Y
Morii
H
H
D000328:Adult; D001706:Biopsy; D003483:Cutis Laxa / Q000150:complications* / Q000276:immunology / Q000473:pathology; D004487:Edema / Q000150:complications / Q000188:drug therapy; D005260:Female; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000150:complications; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D007668:Kidney / Q000473:pathology / Q000648:ultrastructure; D009404:Nephrotic Syndrome / Q000150:complications* / Q000276:immunology; D012867:Skin / Q000473:pathology / Q000648:ultrastructure
D007136:Immunoglobulins