[Acquired generalized cutis laxa with paraproteinemia (IgG lambda). Immunofluorescence study, clinical and histologic findings with review of the literature].
A 49-year-old patient developed acquired generalized cutis laxa, manifest as involvement of the elastic fibres of the dermis and the internal organs, leading to pulmonary emphysema, bilateral inguinal hernia and two oesophageal diverticula. The patient's serum levels of IgG lambda paraprotein were elevated. Direct immunofluorescence examination revealed intensive linear deposits of IgG lambda light chains, an early component of complement C1q and discrete C3 deposits. The deposits were found along the preserved elastic fibres in the dermis, especially in the papillary dermis, at the dermoepidermal junction around the sweat and sebaceous glands and in the walls of small vessels. Dermal alterations of this kind have not been described previously.
Krajnc
I
I
Lehrhospital Maribor, Abteilung für innere Medizin, Slowenien.
Rems
D
D
Vizjak
A
A
Hödl
S
S
D001485:Basement Membrane / Q000276:immunology / Q000473:pathology; D015922:Complement C1q / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D003483:Cutis Laxa / Q000276:immunology / Q000473:pathology*; D004547:Elastic Tissue / Q000473:pathology; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis*; D007146:Immunoglobulin lambda-Chains / Q000032:analysis*; D008875:Middle Aged; D010265:Paraproteinemias / Q000276:immunology / Q000473:pathology*; D012867:Skin / Q000276:immunology / Q000473:pathology; D013545:Sweat Glands / Q000276:immunology / Q000473:pathology
D003176:Complement C3; D007074:Immunoglobulin G; D007146:Immunoglobulin lambda-Chains; D015922:Complement C1q