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Acquired cutis laxa is an uncommon disorder sometimes associated with monoclonal gammopathy and multiple myeloma, although the mechanism of this link is unclear. We report here a case of a 34-year-old man with generalized acquired cutis laxa and monoclonal light chain disease with renal and neurological involvement. Electron microscopy examination of a skin sample revealed shortened and fragmented elastic fibres in the reticular dermis and normal collagen bundles. Immunogold labelling revealed anti-lambda antibodies closely bound to the microfibrillar component of elastic fibres, thus supporting a causal relationship between monoclonal gammopathy and the changes in skin elasticity.

Autoimmune bullous diseases have been reported associated with different causal factors: drugs, mechanical trauma and physical trauma, particularly ultraviolet light and radiotherapy. In these cases different hypotheses regarding the pathogenesis of blister formation can be supported. In this observation, we report a localized cicatricial pemphigoid with unusual clinical presentation. Moreover, it appeared 9 years after radiotherapy for breast carcinoma and it was preferentially localized on an upper limb lymphoedema. Because of the long time between the treatment of carcinoma with radiotherapy and the onset of pemphigoid, we assume that lymphoedema played a major etiological role in this particular cicatricial pemphigoid.

Schnitzler's syndrome, first described in 1974, is defined by chronic non-pruritic urticaria, osteocondentation, and a monoclonal IgM dysproteinemia, but without criteria of lymphoproliferative disease. We report a patient with chronic urticaria and macroglobulinemia. In addition, he had double monoconal dysproteinemia IgM kappa (31.3 g/l) and IgA lambda, osteocondensation, and some cutaneous lesions of pseudoxanthoma elasticum. Only 20 cases of Schnitzler's syndrome have been reported hitherto. This is the first case associated with pseudoxanthoma elasticum, which was localized and discovered at the same time as Schnitzler's syndrome. We discuss the possible role of monoclonal immunoglobulin in the occurrence of localized elastorrexhis.