ACQUIRED GENERALIZED CUTIS-LAXA WITH MONO CLONAL GAMMOPATHY (IGG-LAMBDA) - CLINICAL, HISTOLOGICAL AND IMMUNOFLUORESCENCE EVALUATION AND A REVIEW OF THE LITERATURE

A 49-year-old patient developed acquired generalized cutis laxa, manif est as involvement of the elastic fibres of the dermis and the interna l organs, leading to pulmonary emphysema, bilateral inguinal hernia an d two oesophageal diverticula. The patient's serum levels of IgG lambd a paraprotein were elevated. Direct immunofluorescence examination rev ealed intensive linear deposits of IgG lambda light chains, an early c omponent of complement C1q and discrete C3 deposits. The deposits were found along the preserved elastic fibres in the de-mis, especially in the papillary dermis, at the dermoepidermal junction around the sweat and sebaceous glands and in the walls of small vessels. Dermal altera tions of this kind have not been described previously.