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Krajnc, I; Rems, D; Vizjak, A; Hödl, S.
Acquired generalized cutis laxa with paraproteinemia (IgG lambda). Immunofluorescence study, clinical and histologic findings with review of the literature
Hautarzt. 1996; 47(7):545-549 Doi: 10.1007%2Fs001050050469 (- Case Report)
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Hoedl Stefan
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Abstract:
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.
Find related publications in this database (using NLM MeSH Indexing)
Basement Membrane - immunology
Complement C1q - analysis
Complement C3 - analysis
Cutis Laxa - immunology
Elastic Tissue - pathology
Female - pathology
Fluorescent Antibody Technique, Direct - pathology
Humans - pathology
Immunoglobulin G - analysis
Immunoglobulin lambda-Chains - analysis
Middle Aged - analysis
Paraproteinemias - immunology
Skin - immunology
Sweat Glands - immunology

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