Ultrastructural demonstration of a relationship between acquired cutis laxa and monoclonal gammopathy.
Acquired cutis laxa is an uncommon disorder sometimes associated with monoclonal gammopathy and multiple myeloma, although the mechanism of this link is unclear. We report here a case of a 34-year-old man with generalized acquired cutis laxa and monoclonal light chain disease with renal and neurological involvement. Electron microscopy examination of a skin sample revealed shortened and fragmented elastic fibres in the reticular dermis and normal collagen bundles. Immunogold labelling revealed anti-lambda antibodies closely bound to the microfibrillar component of elastic fibres, thus supporting a causal relationship between monoclonal gammopathy and the changes in skin elasticity.
Maruani
Annabel
A
Department of Dermatology, Université François Rabelais, Tours, France.
Arbeille
Brigitte
B
Machet
Marie-Christine
MC
Barbet
Christelle
C
Laure
Boris
B
Martin
Ludovic
L
Machet
Laurent
L
D000328:Adult; D001323:Autoantibodies / Q000032:analysis; D003094:Collagen / Q000648:ultrastructure; D003483:Cutis Laxa / Q000276:immunology / Q000473:pathology*; D020405:Dermis / Q000276:immunology / Q000648:ultrastructure*; D004547:Elastic Tissue / Q000648:ultrastructure; D006801:Humans; D007146:Immunoglobulin lambda-Chains / Q000276:immunology; D007150:Immunohistochemistry; D008297:Male; D008854:Microscopy, Electron; D010265:Paraproteinemias / Q000276:immunology / Q000473:pathology*
D001323:Autoantibodies; D007146:Immunoglobulin lambda-Chains; D003094:Collagen