Background. Cutis laxa is a rare dermatosis that is inherited or acquired and clinically features loose, wrinkled, and redundant skin\nwith decreased elasticity. This heterogeneous connective tissue disorder may be localized or generalized, with or without internal\nmanifestations. Generalized cutis laxa often has a cephalocaudal progression and is attributed to inflammatory cutaneous\neruptions, medications, and infections. Cutis laxa is also associated with several other conditions including rheumatoid arthritis,\nsystemic lupus erythematosus, and plasma-cell dyscrasias. Case Presentation. We report an unusual case of a 35-year-old male\nwith progression of generalized acquired cutis laxa and vasculitis that occurred over a period of one year. No cutaneous inflammatory\neruption preceded or accompanied his decreased skin elasticity, and a biopsy of the skin showed elastolysis. His\ncutaneous manifestation led to systemic evaluation and an eventual diagnosis of smoldering multiple myeloma accompanied by\naortitis and anemia. His myeloma and vasculitis were successfully treated with cyclophosphamide, bortezomib, and dexamethasone\nand high-dose prednisone, respectively, with no improvement to his cutis laxa. Conclusions. The presence of\nmonoclonal gammopathy is strongly associated with several dermatological entities such as acquired cutis laxa. We propose a new\nterm for the dermatological manifestations caused by paraproteinemia: monoclonal gammopathy of dermatological significance,\nor MGODS, and stress the evaluation of an underlying gammopathy in the setting of certain dermatologic conditions, including\nscleromyxedema and amyloidosis. We present a case of a newly acquired cutis laxa secondary to plasma-cell dyscrasias that\nexemplifies MGODS, alongside a brief literature review, and underscore the clinical relevance of monoclonal gammopathies of\ndermatological significance.
Loading....