Erworbene generalisierte Cutis laxa mit Paraproteinämie (IgG-lambda)

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.

Clinical Presentation of a Patient with Localized Acquired Cutis Laxa of Abdomen: A Case Report

“…Occasionally a scant, but not diagnostic, perivascular lymphohistiocytic infiltrate is seen. There was no epidermal alteration or cellular inflammatory infiltrate and also no evidence of IgG and IgA deposits in lesional skin which could, as in the case report of acquired CL described by Krajnc et al, lead to conclusion that, in our case, immunopathogenetic mechanisms may not play a role in the loss of elastin fibres [7]. …”

“…The enhanced degradation of the elastic fibers is a possible mechanism for the development of acquired localized CL due to powerful proteolytic elastases which are released from inflammatory cells, such as polymorphonuclear leukocytes and monocyte-macrophages into extracellular matrix [6]. Furthermore, immunopathogenetic mechanisms may play a role in a small subset of patients, as evidenced by IgG and IgA deposits in lesional skin or paraproteinemia [7]. There may be an underlying genetic susceptibility for the development of acquired CL, for example, missense alleles in the elastin and fibulin-5-genes [8, 9].…”

Cutis Laxa: Improvement of Facial Aesthetics by Using Botulinum Toxin

“…CUTIS LAXA, also called generalized elastosis or dermatochalasis, is a rare disease characterized by the total loss of skin elasticity that leads to the appearance of early aging. They can be inherited or secondary to previous inflammatory skin alterations 1–14 . Histologically there is a progressive destruction of the dermal elastic fiber 15–23 …”