Acquired cutis laxa associated with a plasma cell dyscrasia.
Acquired cutis laxa (generalized acquired elastolysis) is characterized by skin laxity often with systemic involvement of the lungs, aorta, gastrointestinal tract, and pelvic organs. Although there have been many speculations regarding its pathogenesis, the etiology of this condition is still unclear. We describe a patient with generalized acquired cutis laxa associated with a plasma cell dyscrasia. Immunofluorescence studies were used to evaluate the pathogenesis of the elastolysis. Lesional skin was examined by direct and indirect immunofluorescence techniques for evidence of deposition of immunoglobulins on elastic fibers in the dermis. Direct immunofluorescence revealed deposition of IgG on elastic fibers in the dermis. Some patients with acquired cutis laxa have underlying lymphoreticular disorders such as plasma cell dyscrasia and may have immunoglobulins deposited on dermal elastic fibers. Immune-mediated mechanisms may play a major role in the pathogenesis of acquired cutis laxa in at least some cases.
Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, USA.
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A
Nikko
M
M
Dunnigan
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A
Black
C J
CJ
Cockerell
D000328:Adult; D003483:Cutis Laxa / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D010265:Paraproteinemias / Q000150:complications* / Q000473:pathology
D007074:Immunoglobulin G